ANSWERS 21 - 40

21.Fungal infections of the scalp. Etiology (pathogens), clinical signs, diagnostic, local and systemic treatment.

It is a worldwide well-known superficial fungal infection of the scalp hair with varied clinical manifestation. Children up to puberty are susceptible. The infection is transmitted by direct contact and through fomites such as combs, hairbrushes, barbers’ instruments and hats. A break in cutaneous barrier is essential to inoculate the fungus.

ETIOLOGY-

ringworm and tonsurans infection in cutaneous of scalp.The disease is primarily caused bydermatophytes in the Trichophyton and Microsporum genera that invade the hair shaft.

(M. canis, M. audouinii. T. tonsurans, T. Schoenleinii)

CLINICAL SINGS-

The clinical picture usually varies according to the causative dermatophyte. Some strains such as Microsporon Canis and T. Mentagrophtes cause highly inflammatory lesions, while T. Tonsurans lesions have a very chronic course. The clinical picture may be sometimes confusing and cannot be easily diagnosed except by detection of the dermatophyte by potassium hydroxide smears.

Different clinical types of Tinea Capitis 

 Dry Type - lesion may be dry and scaly simulating dandruff of the scalp, psoriasis and lichen planus. 

 Black Dot Type - usually the lesion is dry where the hair is cut short from the stumps, and the bases of infected hair are prominent. There is a variable degree of erythema, itching and scaling. The individual lesion may persist for a long time or resolve spontaneously.

 Kerion - Other lesions may be highly inflammatory which show swollen, edematous, oozing and crusting lesion in the form of boggy inflammation of the scalp called "kerion". This type may be mis-diagnosed and treated as an abscess of the scalp. Hair loss may be permanent causing cicatricial alopecia.

  Favus - the clinical picture of favus is characteristic where solid crust is formed on the infected area, which may spread to cover the whole scalp. The scalp has special mouse smell. The condition is very chronic and may end with cicatricial alopecia. The infection may spread to other areas away from the scalp such as to the abdomen and extremities

DIAGNOSIS-

The clinical picture - fungi causing T. capitis characteristically begin the pathological manifestations in the center feeding on the keratin and spreading peripherally away from the center. There is central clearing where the periphery of the lesion shows active edges either papular, vesicular or papulovesicular edge with scaling surface.

Wood‘s light - microsporon gives strong green fluorescence. Trichophyton groups such as T. Schoenleini give dull green fluorescence under a filtered ultra violet, Wood‘ light in a dark room. This is very helpful in rapid screening of large number of school children. 

Smear - this is a simple method and can be done easily in the office. Microscopic examination of the specimen by potassium hydroxide smears can detect the hyphae of the causative dermatophyte. Technique: Collection of scrapings from the infected skin should be taken from the active edge of the lesion using a blunt scalpel blade or by the edge of a slide. Infected hair should be depilated from its roots especially in favus.

The specimen is placed on a slide and a drop of 30 percent potassium hydroxide is added and covered by a cover slip. It is heated gently in order to soften and clear the material . Care should be taken in order not to heat the specimen too much and not to boil. The specimen is placed on a slide and a drop of 30 percent potassium hydroxide is added and covered by a cover slip . It is heated gently in order to soften and clear the material . Care should be taken in order not to heat the specimen too much and not to boil . 

Culture - Culture is on petri dishes or cotton wool - plugged test tubes with Sabouraud‘s dextrose agar containing antibiotics to inhibit bacterial and saprophyte contamination. Incubation is kept at 26-30 C for one to two weeks. Different colonies can be identified morphologically and microscopically

LOCAL AND SYSTEMIC THERAPY-

systemic-

Griseofulvin,Infants and children : • 125mg/day up to the age of 1 year (one teaspoonful ). • 187mg/day from 1 to 5 years (one and a half teaspoonful), and. • 250-375mg/day (2-3 teaspoonful) from 6 to 12 years divided into two doses or as one dose after a fatty meal.

In children the daily dose is 10mg/kg/day given in two divided doses daily. It should be after meals (after fatty meal as after eating an egg). The duration of treatment varies from ten to twenty days according to the type and severity of the fungal infection . Adult dose : • 0ne to two 500mg tablet daily or at least 10 mg/ kg /day. • Small adults (55 kg). one tablet 250 mg. twice daily. • Medium-sized adults, one tablet 250 mg. three times daily. • For large adults (over 100-kg), one tablet 500 mg. twice daily).

Azoles

Azoles Itraconazole - these are effective new anti-fungal preparations. Dose: Adult: 100-200mg. /day for few weeks in skin fungal infection and for several months in onychomycosis.( Side effects of itraconazole: • Gastro-intestinal disturbances . • Headache. • Rarely exfoliative dermatitis ).

Ketoconazole: This orally active imidazole is a broad-spectrum anti-fungal agent. Dose: Adult: 200-400 mg/day with food and is usually well tolerated. (Side effects: Headache and nausea are relatively common minor side effects.)

 Ketoconazole may inhibit androgen biosynthesis. Liver enzymes should be measured at monthly intervals with prolonged courses . Treatment should be stopped if ALT or SGPT rise two- to threefold.

The two main compounds are Terbinafine and Naftifine. Both are active against dermatophytes. Terbinafine such as Lamasil can be given orally. Dose: Children above 20kg can be given 62.5 mg daily. 20- 40-kg-body weight : 125 mg can be given daily . Above 40 kg - 250 mg daily. The adult dose is 250 mg daily. Terbinafine is available also as topical preparation (Lamasil cream). It has produced rapid and long-lasting remissions in both nail diseases and persistent Tinea pedais.

Voriconazole: The anti-fungal agent voriconazole is well tolerated, with only mild to moderate adverse effects, report researchers. (The most common of side effects are headache, rash, abnormal vision)

LOCAL-

Terbinafine (lamasil cream)

22.Onychomycosis. Etiology (pathogens), clinical signs, diagnostic, local and systemic treatment

The course of the disease is chronic. The lesion manifests with yellowish discoloration of the nail tip which may spread to involve the whole nail. The nail color is changed and shows dirty debris underneath. Later the nail becomes brittle and breaks off leaving undermined black remnants.

ETIOLOGY-

• T. Rubrum : Causes chronic infection with little inflammatory reaction. 

• T. Mentagrophtes infection: Causes superficial and usually localized nail infection. 

• Candidal nail infection: The disease is usually mild and begins on the nail fold. The adjacent cuticle is pink, swollen, and tender and characteristically, beads like pus can be expressed from the lesion. The affected nail may become dark, ridged and may become separated from its nail bed.

CLINICAL SIGN-

The most common symptom of a fungal nail infection is the nail becoming thickened and discoloured: white, black, yellow or green. As the infection progresses the nail can become brittle, with pieces breaking off or coming away from the toe or finger completely. If left untreated, the skin can become inflamed and painful underneath and around the nail. There may also be white or yellow patches on the nailbed or scaly skin next to the nail,and a foul smell.There is usually no pain or other bodily symptoms, unless the disease is severe.
         People with onychomycosis may experience significant psychosocial problems due to the appearance of the nail, particularly when fingers – which are always visible – rather than toenails are affected.

         Dermatophytids are fungus-free skin lesions that sometimes form as a result of a fungus infection in another part of the body. This could take the form of a rash or itch in an area of the body that is not infected with the fungus. Dermatophytids can be thought of as an allergic reaction to the fungus.

DIAGNOSIS-

To avoid misdiagnosis as nail psoriasis, lichen planus, contact dermatitis, nail bed tumors such as melanoma, trauma, or yellow nail syndrome, laboratory confirmation may be necessary.
The three main approaches are potassium hydroxide smear, culture and histology.This involves microscopic examination and culture of nail scrapings or clippings. Recent results indicate the most sensitive diagnostic approaches are direct smear combined with histological examination, and nail plate biopsy using periodic acid-Schiff stain.To reliably identify nondermatophyte molds, several samples may be necessary.

SYSTEIMIC AND LOCAL TREATMENT-

Griseofluvin, pediatric dosage is 10 mg/kg/day for 6–8 weeks, although this may be increased to 20 mg/kg/d for those infected by T. tonsurans, or those who fail to respond to the initial 6 weeks of treatment.

terbinafine, itraconazole, and fluconazole. Oral dose.

Griseofluvin is used  in topical way too.

23.Tinea Versicolor. Etiology, clinical features, diagnostic, treatment.

ETIOLOGY-

    Malassezia globosa fungus, although Malassezia furfur is responsible for a small number of cases.These yeasts are normally found on the human skin and only become troublesome under certain circumstances, such as a warm and humid environment.

CLINICAL FEATURES-

begins as light brown patches or hypopigmented macules of 5 mm to 20 mm in diameter, most frequently on the trunk, but sometimes on the upper arms and neck. They gradually enlarge and coalesce, presenting larger macules.

tinea versicolor in which brown patches are produced is called tinea versicolor nigra; tinea versicolor in which hypopigmented macules occur is called  tinea versicolor alba. The patches tend to be asymptomatic, although there  may be mild reddening or itching.

DIAGNOSIS-

  Diagnosis of pityriasis versicolor is confirmed by clinical features, KOH direct microscopy and fluorescence under Wood’s lamp.

TREATMENT-  

heals relatively easily with topical imidazole antifungal agents in about two weeks. It is both chronic and recurrent

24.Candidiasis. Etiology, clinical features, diagnostic, treatment.

It is an infection of the skin or mucous membrane caused by yeasts of the genus Candida.Candidiasis in dermatology is classified by location and clinical features into three main subtypes: cutaneous, mucosal and atypical

Cutaneous candidiasis is a superficial infection occurring on moist cutaneous sites;( many patients have predisposing factors that alter local immunity such as increased moisture at the site of infection, diabetes, or alteration in systemic immunity.)

Mucosal candidiasis is a Candida infection occurring on the mucosa of the upper aerodigestive tract and vulvovagina; the course may be acute or chronic.

ETIOLOGY-

There are seven to ten virulent species in the genus Candida. The main causative species is known to be Candida albicans.

For Cutaneous candidiasis : C. albicans is seldom recovered from skin of normal individuals.

For mucosal candidiasis-

C. albicans, most commonly, but also C. tropicalis, C. (Torula) glabrata, C. Parapsilosis, C. krusei, C. lusitaniae, C. rugosa. In some individuals, two or more species can be isolated.

 CLINICAL FEATURES-

For cutaneous candidiasis:

ü  Intertrigo -Initial pustules on erythematous base become eroded and confluent. Subsequently, fairly sharply demarcated, polycyclic, erythematous, eroded patches with small pustular lesions at the periphery (satellite pustulosis). Distribution: inframammary , axillae, groins , perineal, intergluteal cleft.

ü  Interdigital Erosio interdigitalis blastomycetica: Initial pustule becomes eroded, with formation of superficial erosion or fissure, 6surrounded by thickened white skin .

ü  Balanoposthitis, Balanitis Preputial sac: pustules, erosions . Maculopapular lesions with diffuse erythema.Edema, ulcerations, and fissuring of prepuce, usually in diabetic men; white plaques under foreskin. Vulvitis Erosions, pustules, erythema , swelling, removable curdlike material Diaper Dermatitis Erythema, edema with papular and pustular lesions; erosions, oozing, collarette-like scaling at the margins of lesions involving perigenital and perianal skin, inner aspects of thighs and buttock

ü  Candida Paronychia and Onychia Initially, redness and swelling of proximal (also lateral) nail folds. Swelling lifts wall from underlying nail plate. Subsequently, infection becomes purulent, pressure releases creamy pus from nail fold . Painful. Nails may show onychodystrophy, onycholysis, discoloration (yellow, green, or black on lateral nail), and ridging.

For mucosal candidiasis:

Skin Lesions

TYPES

ü  VC Vaginitis

-with white discharge; vaginal erythema and edema; white plaques that can be wiped off on vaginal and/or cervical mucosa.

ü  OPC

• Pseudomembranous candidiasis (thrush)  removable white plaques on any mucosal surface; vary in size from 1 to 2 mm to extensive and widespread; removal with a dry gauze pad leaves an erythematous or bleeding mucosal surface.

• Erythematous (atrophic) smooth, red, atrophic patches

• Candidal leukoplakia: white plaques that cannot be wiped off but regress with prolonged anticandidal therapy

• Angular cheilitis: erythema, fissuring, i.e., intertrigo at the corner of mouth.

COLOR Thrush: white to creamy

PALPATION Thrush: removable with dry gauze

DIAGNOSIS-

Cutaneous candidiasis-Clinical findings confirmed by direct microscopy or culture.

Mucosal candidiasis-clinical suspicion confirmed by KOH preparation of scraping from mucosal surface.

TREATMENT-

Cutaneous candidiasis-

Short-term use of topical corticosteroid preparation speeds resolution of symptoms.

INTERTRIGO Castellani’s paint. Nystatin or imidazole creams applied.

BALANITIS/BALANOPOSTHITIS, VULVITIS Castellani’s paint.Nystatin or imidazole creams applied

VULVITIS Castellani’s paint, nystatin, azole, or imidazole creams applied (vaginal candidiasis).

DIAPER CANDIDIASIS Dilute Castellani’s paint. Nystatin or imidazole creams applied ,Oral nystatin (which is not absorbed from the gut) eliminates bowel candidal colonization, thus reducing frequent recurrences.

PARONYCHIA Castellani’s paint. Eliminate immersion in water if possible. Nystatin or imidazole creams applied  If topical therapy fails, systemic treatment is effective: fluconazole 200 mg weekly or itraconazole 200 mg/day until space between nail fold and nail plate has been eliminated.

Mucosal candidiasis-Butoconazole:

VC: Butoconazole: 2 % cream  Clotrimazole: 1% cream 5 g

 Miconazole: 2% cream 5g

  Tioconazole: 6.5% ointment 5 g or Terconazole: 0.4% cream 5 g

 Fluconazole: 150 mg.

OPC TOPICAL THERAPY These preparations are effective in the immunocompetent individual but relatively ineffective with decreasing cell-mediated immunity. Nystatin: For OPC, vaginal tablets, 100,000 units . dissolved slowly in the mouth, are the most effective preparation.

 The oral suspension, 1 to 2 teaspoons, held in mouth for 5 minutes and then swallowed may be effective. Clotrimazole: For OPC, oral tablets (troche), 10 mg, one tablet 5 times daily may be effective.

  

25. Lyme disease: skin lesions, lab tests, treatment.

Lyme disease is a cutaneous and systemic infection caused by the spirochaete Borrelia burgdorferi and spread by tick-bite

SKIN LESIONS

TYPES OF LESIONS

 Erythema Migrans (EM) Initial macule or papule enlarges within days to form an expanding annular lesion with a distinct red border and partially clearing middle, i.e., EM, at the bite site. Maximum median diameter is 15 cm (range 3 to 68 cm). Center may become indurated, vesicular, or necrotic. At times concentric rings form. When occurring on the scalp, only a linear streak may be evident on the face or neck. Multiple EM lesions are seen when multiple bite sites occur.

 Secondary Lesions 17 % develop multiple annular secondary lesions, ranging in number from 2 to >100. Secondary lesions resemble EM but are smaller, migrate less, and lack central induration; 17 % of patients have multiple secondary EM lesions ranging in number from 2 to 36.

 Lymphocytoma Cutis (LC) Synonyms: Lymphadenosis benigna cutis (LABC), pseudolymphoma of Spiegler and Fendt. Most often a solitary (may be grouped) nodule or plaque; occasionally translucent; red to brown to purple in color; located on the head, especially earlobe, areola, scrotum, and extremities; 3 to 5 cm in diameter; usually asymptomatic. Systemic Bacterial Infections: Lyme Borreliosis body regions subject index table of contents

Other Cutaneous Findings Malar rash, diffuse urticaria, subcutaneous nodules (panniculitis)

COLOR OF LESIONS EM: Erythematous evolving to violaceous

DISTRIBUTION OF LESIONS EM: Trunk and proximal extremities, especially the axillary and inguinal areas, most common sites. Secondary lesions: any site except the palms and soles; can become confluent.

MUCOUS MEMBRANES Red throat, conjunctivitis

LB may occur without EM or secondary lesions and present only with the late manifestations. Also, late manifestations may occur despite treatment (inadequate) of early LB with tetracycline

LAB TESTS

Skin Biopsy

Serology: A two-test approach for active disease and for previous infection using a sensitive enzyme immunoassay (EIA) or immunofluorescent assay (IFA) followed by a Western immunoblot (WIB) is recommended

Culture B. burgdorferi can be isolated from lesional skin biopsy specimen on Kelly’s medium.

PCR Detects B. burgdorferi DNA in lesional skin biopsy specimen, blood, or joint fluid. May be the preferred confirmatory laboratory test.

TREATMENT.

Prophylaxis

Avoid known tick habitats. Other preventive measures include wearing long pants and long-sleeved shirts, tucking pants into socks, applying tick repellents containing N,N-diethyl-m-toluamide (“DEET”) to clothing and/or exposed skin, checking regularly for ticks, and promptly removing any attached ticks. Acaracides containing permethrin kill ticks on contact and can provide further protection when applied to clothing.

Antimicrobial Treatment

 EARLY LYME BORRELIOSIS Without neurologic, cardiac, or joint involvement

 • Amoxicillin 500 mg PO t.i.d. for 21 days (if only EM, 10 days sufficient)

 • Cefuroxime axetil 500 mg PO q.d. for 7 days

 • Doxycycline 100 mg PO b.i.d. for 21 days (if only EM, 10 days sufficient)

• Azithromycin 500 mg PO q.d. for 7 days (less effective)

26. Urticaria. Etiopathogenesis, clinical forms, diagnostic, treatment,

Urticaria (hives) is a common eruption characterized by transient usually pruritic, wheals due to acute dermal oedema from extravascular leakage of plasma.

ETIOPATHOGENESIS:

Urticaria is mediated through immune (allergic) or non-immune mechanisms. Lesions result from the release from mast cells of biologically active substances, particularly histamine, which produce vasodilatation and increased vascular permeability.

CLINICAL FORMS

1) Acute and chronic urticaria Urticaria is divided by duration of episode into acute (less than 6 weeks) and chronic (6 weeks or longer).

2) Contact urticaria Contact urticaria occurs at sites where a foreign substance comes into contact with and permeates the skin or mucosa. It is classified into allergic contact urticaria and nonallergic contact urticaria. Patients with allergic contact urticaria have had previous contact with the substance and are sensitized to it. Nonallergic contact urticaria is caused by the first contact with a substance (MEMO).

 3) Physical urticaria Physical urticaria is an eruption caused by physical stimulation. It disappears in 30 minutes to 1 hour. It is divided into several subtypes according to cause. Factitious urticaria, also called mechanical urticaria, is produced by rubbing. Dermographism is positive in factitious urticaria. Solar urticaria is caused by sunlight. Cold urticaria is caused by exposure to the cold, such as cold water or wind.

 4) Cholinergic urticaria Cholinergic urticaria occurs when the body perspires after the body temperature rises from exercise or bathing . The cholinergic nerves are thought to be involved

DIAGNOSTIC

It is easy to diagnose urticaria by the clinical findings. Historytaking on suspected causative agents of urticaria, such as mechanical stimuli, the cold, foods and drugs, is helpful. Since urticaria sometimes accompanies systemic diseases, including collagen diseases, determination of the primary disease is necessary. Dermographism is positive (when the skin is rubbed, the site turns red; Tests such as that for serum IgE levels, IgE RAST (radioallergosorbent test), intradermal allergic test and drug-induction test are conducted.

TREATMENT

Prevention Try to prevent attacks by elimination of etiologic chemicals or drugs: aspirin and food additives, especially in chronic recurrent urticaria—rarely successful.

Antihistamines H1 blockers, e.g., hydroxyzine, terfenadine; and if they fail, H1 and H2 blockers (cimetidine) and/or mast cell stabilizing agents (ketotifen). Doxepin, a tricyclic antidepressant with marked H1 antihistaminic activity, is valuable when severe urticaria is associated with anxiety and depression.

Prednisone Indicated for angioedema-urticaria-eosinophilia syndrome.

 Danazol Long-term therapy for hereditary angioedema; whole fresh plasma or C1 esterase inhibitor in the acute attack

27. Eczema. etiopathogenesis, clinical signs, diagnostic, treatment.

Eczema is a non-infective inflammatory condition of the skin. The term 'eczema' literally means 'to boil over' (Greek), and this well describes the acute eruption in which blistering occurs.

ETIOPATHOGENESIS-

Pathologically, eczema is accompanied by itching, reddening, scaling, and edematous or serous papules. Histopathologically, it is characterized by intercellular edema (also called spongiosis)

Extrinsic and intrinsic factors are simultaneously involved in its onset.

Both extrinsic and intrinsic factors are involved in eczema. When an extrinsic agent such as a drug, pollen, house dust, or bacteria invades the skin, an inflammatory reaction is induced to eliminate the foreign substance. The severity and type of reaction vary according to intrinsic factors such as seborrhea, dyshidrosis, atopic diathesis, and the health condition of the patient.

CLINICAL SIGN-

Itchy edematous erythema forms, on which papules and serous papules are produced. After the formation of vesicles, pustules, erosions, crusts and scales, the condition begins to subside.

In the acute stage, these symptoms are present singly or together. In the chronic stage, acanthosis, lichenification, pigmentation and depigmentation are found, in addition to the symptoms of the acute stage.

DIAGNOSIS-

Diagnosis of eczema is based mostly on the history and physical examination.However, in uncertain cases, skin biopsy may be useful. Those with eczema may be especially prone to misdiagnosis of food allergies.^.

^{Patch test}

^{General blood test}

^{Serological test}

  

TREATMENT-

There is no known cure for eczema, with treatment aiming to control symptoms by reducing inflammation and relieving itching.

Ø  Moisturizing agents (also known as emollients) are recommended at least once or twice a day.Oilier formulations appear to be better and water-based formulations are not recommended.It is unclear if moisturizers that contain ceramides are more or less effective than others.Products that contain dyes, perfumes, or peanuts should not be used.Occlusive dressings at night may be useful.

Ø  Medications used for Antihistamines ,diphenhydramine.

Ø  Corticosteroids are effective in controlling and suppressing symptoms in most cases.

    For mild-moderate eczema a weak steroid may be used (e.g. hydrocortisone).

Ø  Immunosuppressants-

    The most commonly used are ciclosporin,azathioprine, and methotrexate.

28. Allergic contact dermatitis: etiopathogenesis, clinical signs, diagnostic, treatment.

An adverse cutaneous inflammation reaction caused by contact with a specific exogenous allergen to which a person has developed allergic sensitization.

ETIOPATHOGENESIS

Contact (allergic) dermatitis, more commonly called contact eczematous dermatitis, is a classic, delayed, cell-mediated hypersensitivity reaction. Exposure to a strong sensitizer such as poison ivy resin results in a sensitization in a week or so, while exposure to a weak allergen may take months to years for sensitization.

COMMON CONTACT ALLERGENS

 Neomycin                      Usually contained in creams, ointments

Procaine, benzocaine      Local anesthetics

Sulfonamides

Terpentine                       Solvents, shoe polish, printer’s ink

Balsam of Peru                Topical

Thiuram                           Rubber

Formalin                          Disinfectant, curing agents, plastics

Mercury                           Disinfectant, impregnation

Chromates                        Cement, antioxidants, industrial oils, matches

Nickel sulfate                   Metals, metals in clothing, jewelry, catalyzing agents

Cobalt sulfate                   Cement, galvanization, industrial oils, cooling agents, eye shades

p-Phenylene diamine        Black or dark dyes of textiles, printer’s ink Parahydroxybenzoic acid ester Conserving           

CLINICAL SIGNS

Duration of Lesion(s) Acute contact—days, weeks; chronic contact—months, years

Skin Symptoms Pruritus

Constitutional Symptoms “Acute illness” syndrome (more info) (including fever) in severe allergic contact dermatitis (e.g., poison ivy)

Skin Lesions

TYPE Acute Well-demarcated plaques of erythema and edema on which are superimposed closely spaced, nonumbilicated vesicles, punctate erosions exuding serum, and crusts

Subacute Plaques of mild erythema showing small, dry scales or superficial desquamation, sometimes associated with small, red, pointed or rounded, firm papules  

Chronic Plaques of lichenification (thickening of the epidermis with deepening of the skin lines in parallel or rhomboidal pattern), with satellite, small, firm, rounded or flat-topped papules, excoriations, and pigmentation or mild erythema  

ARRANGEMENT Often linear, with artificial patterns, an “outside job”. Plant contact often results in linear lesions.

DISTRIBUTION Extent Isolated, localized to one region (e.g., shoe dermatitis), or generalized (e.g., plant dermatitis)

Pattern Random or on exposed areas (as in airborne allergic contact dermatitis)

DIAGNOSTIC

1.     Patch test

2.     Skin biopsy

3.     Culture of skin lesion

4.     General blood test

5.     Immunological test

6.     Culture of skin lesion

TREATMENT

Acute

 Identify and remove the etiologic agent.

Larger vesicles may be drained, but tops should not be removed.

Wet dressings using gauze soaked in Burow’s solution changed every 2 to 3 hours

Topical class I corticosteroid preparations

In severe cases, systemic corticosteroids may be indicated.

Prednisone: two-week course, 70 mg initially, tapering by 5 mg daily

Subacute and Chronic If the lesions are not bullous, it is possible to use a short course of one of the potent topical corticosteroid preparations, betamethasone dipropionate or clobetasol propionate.

29. Erythema nodosum: etiopathogenesis, clinical signs, diagnostic, treatment.

Erythema nodosum is a panniculitis (i.e. an inflammation of the subcutaneous fat) that usually presents as painful red nodules on the lower

legs.

ETIOPATHOGENESIS

It is believed to result from CIC deposition in vessels of the subcutis. Infection, drugs and some systemic diseases are underlying causes

Etiologic Associations

INFECTIOUS AGENTS Rarely primary tuberculosis (children), coccidioidomycosis, histoplasmosis, beta-hemolytic streptococcus, Yersinia organisms, lymphogranuloma venereum, leprosy (erythema nodosum leprosum)

DRUGS Sulfonamides, oral contraceptives

MISCELLANEOUS Sarcoidosis (quite often), ulcerative colitis, Behçet’s syndrome, idiopathic 640 %

CLINICAL SIGNS

Age 15 to 30 years, but age distribution related to etiology

Sex Three times more common in females than in males

Duration of Lesions Days

Skin Symptoms Painful, tender lesions

Constitutional Symptoms Fever, malaise

Systems Review Arthralgia (50 %) most frequently of ankle joints, other symptoms depending on etiology

Skin Lesions

TYPE: Nodules (3.0 to 20.0 cm) not sharply marginated, deep-seated. Nodules are located in the subcutaneous fat and are appreciated as such only upon palpation. The term erythema nodosum best describes the skin lesions: they look like erythema but feel like nodules.

COLOR Bright to deep red, later violaceous and even later, brownish, yellow-green, like resolving hematomas.

PALPATION Indurated, tender

SHAPE Oval, round, arciform

ARRANGEMENT Scattered discrete lesions (2 to 50)

DISTRIBUTION Bilateral but not symmetric

SITES OF PREDILECTION Lower legs (most frequently), knees, arms, rarely face and neck

DIAGNOSIS

Clinical signs

Bacterial Culture Culture throat for GABHS (group A beta-hemolytic streptococcus), stool for Yersinia. Imaging

Radiologic examination of the chest is important to rule out sarcoidosis.

TREATMENT

Symptomatic Bed rest or compressive bandages (lower legs).

 Anti-inflammatory Treatment Salicylates, NSAIDs.

Prednisone Response to systemic corticosteroids is rapid but their use is indicated only when the etiology is known (and infectious agents are excluded).

30. Erythema exsudativum multiforme: clinical signs, diagnostic, treatment.

CLINICAL SIGNS

Age 20 to 30 years, with 50 % under 20 years

Sex More frequent in males than in females

Etiologies DRUGS Sulfonamides, phenytoin, barbiturates, phenylbutazone, penicillin, allopurinol

                  INFECTION Especially following herpes simplex, Mycoplasma

                   IDIOPATHIC > 50 %

Duration of Lesions Several days. May have history of prior episode of erythema multiforme (EM).

Skin Symptoms May be pruritic or painful

Mucous Membrane Symptoms Mouth lesions are painful, tender.

Constitutional Symptoms Fever, weakness, malaise

Skin Lesions Lesions may develop over 10 days or more.

 TYPE

Macule (48 hours) → papule, 1.0 to 2.0 cm; lesions may appear for 2 weeks. Vesicles and bullae (in the center of the papule)

 COLOR Dull red

SHAPE Iris or target lesions are typical  

ARRANGEMENT Localized to hands or generalized

DISTRIBUTION Bilateral and often symmetric

SITES OF PREDILECTION Dorsa of hands, palms, and soles; forearms; feet; face; elbows and knees; penis (50 %) and vulva (see Figure IX)

Mucous Membranes Erosions with fibrin membranes: lips, oropharynx, nasal, conjunctival, vulvar, anal

Other Organs Pulmonary, eyes with corneal ulcers, anterior uveitis

DIAGNOSIS

The target lesion and the symmetry are quite typical, and the diagnosis is not difficult. In the absence of skin lesions, the mucous membrane lesions may present a difficult differential diagnosis: bullous diseases, fixed drug eruption, and primary herpetic gingivostomatitis. Urticaria.

TREATMENT

Prevention Control of herpes simplex using oral acyclovir may prevent development of recurrent erythema multiforme. Corticosteroids In severely ill patients, systemic corticosteroids are usually given (prednisone 50 to 80 mg daily in divided doses, quickly tapered), but their effectiveness has not been established by controlled studies.

31. Atopic Dermatitis. Etiopathogenesis, clinical features, diagnostic, local and systemic treatment, physiotherapy.

Atopic dermatitis (AD) is an acute, subacute, but usually chronic pruritic inflammation of the epidermis and dermis, often occurring in association with a personal or family history of hay fever, asthma, allergic rhinitis, or atopic dermatitis. Definition of atopy: literally, “no (without) place.” A heritable clinical state associated with dermatitis, asthma, and allergic rhinitis.

Synonyms: “Eczema,” atopic eczema, IgE dermatitis

ETIOPATHOGENESIS

Atopy1 defines an inherited tendency, present in 15-25% of the population, to develop one or more of the aforementioned disorders and to

produce high levels of circulating IgE antibodies, commonly to inhalant allergens (e.g. house dust mite). A Th2 cell response seems to be predominant over Thl  and the resultant cytokine profile favours IgE production. Notwithstanding this, there is evidence that Thl cell immunity directed against housedust mite antigen may be important in atopic eczema, although the basic cause of these immune defects

is still unclear.

CLINICAL FEATURES

Age Onset in first 2 months of life and by first year in 60 % of patients

Sex Slightly more common in males than in females

The appearance of atopic eczema differs depending on the age of the patient.

Infancy

Babies develop an itchy vesicular exudative eczema on the face and hands, often with secondary infection. Less than half continue to have eczema beyond 18 months.

Childhood

After 18 months, the pattern often changes to the familiar involvement of the antecubital and popliteal fossae, neck, wrists and ankles. The face often shows erythema and infraorbital folds. Lichenification, excoriations and dry skin  are common, and palmar markings may be increased. Post-inflammatory hyperpigmentation occurs in those with a pigmented skin. Scratching or rubbing cause most of the clinical signs and are particularly a problem at night when they can interfere with sleep. Hyperactivity is sometimes seen, and the child may use his or her eczema to manipulate the family. Schoolchildren with eczema may be teased or rejected by their classmates.

Adults

The commonest manifestation in adult life is hand dermatitis, exacerbated by irritants, in someone with a past history of atopic eczema. However, a small number of adults have a chronic severe form of generalized and lichenified atopic eczema which may interfere with their employment and social activities. Stressful situations, such as examinations or marital problems, often coincide with exacerbations.

DIAGNOSIS

History in infancy, clinical findings (typical distribution sites, morphology of lesions, white dermographism)

TREATMENT

Education of the patient to avoid rubbing and scratching is most important. Topical preparations are valuable but are useless if the patient continues to scratch and rub the plaques.

An allergic workup is rarely helpful in uncovering an allergen; however, in patients who are hypersensitive to house dust, mites, various pollens, and animal hair proteins, exposure to the appropriate allergen may cause flares. Atopic dermatitis is considered by many to be related, at least in part, to emotional stress. Education of the patient to avoid rubbing and scratching is most important. Topical antipruritic (menthol/camphor) lotions are helpful in controlling the pruritus.

Warn patients of their special problems with herpes simplex and the frequency of superimposed staphylococcal infection, for which oral antibiotics are indicated. Acyclovir is indicated if HSV infection is suspected.

Acute

1. Wet dressings and topical corticosteroids; topical antibiotics (mupirocin ointment) when indicated

2. Oral H1 antihistamines for pruritus

3. Oral antibiotics (cloxacillin, erythromycin) to eliminate staphylococci

Subacute and Chronic

 1. Oral H1 antihistamines are useful in reducing itching.

2. Topical ointments containing H1 and H2 blockers (doxepin) are only rarely useful for pruritus.

3. Hydration (oilated baths or baths with oatmeal powder) followed by application of unscented emollients (e.g., hydrated petrolatum) is a basic daily treatment needed to prevent xerosis. Soap showers are permissible in order to wash the body folds, but soap seldom should be used on the other parts of the skin surface. 12 % ammonium lactate or 10 % alpha hydroxy acid lotion is very effective for the xerosis often seen in AD.

4. Topical anti-inflammatory agents such as corticosteroids, hydroxyquinoline preparations, and tar are the mainstays of treatment. Of these, corticosteroids are the most readily accepted by the patient.

5. Systemic corticosteroids should be avoided, except in rare instances for only short courses. For severe intractable disease, prednisone 70 mg tapered by 5 mg/day. Corticosteroids should be given with meals and in divided doses. AD patients tend to become dependent on oral corticosteroids. Often small doses (5 to 10 mg) will make the difference in control and can be reduced gradually, to even 2.5 mg daily.

6. Cyclic administration (e.g., every 2 or 3 weeks) for 5 to 6 days of erythromycin or other antibiotic to combat staphylococcal colonization.

32. Psoriasis. Etiopathogenesis, clinical forms, clinical signs, diagnostic, local and systemic treatment, physiotherapy.

Psoriasis is a chronic, non-infectious inflammatory dermatosis characterized by well-demarcated erythematous plaques topped by silvery scales

ETIOPATHOGENESIS

Unknown etiology

 Genetics

Precipitating factors

 A number of precipitating factors are associated with the disorder:

 « Koebner phenomenon. Trauma to the epidermis and dermis, such as a scratch or surgical scar, can precipitate psoriasis in the damaged skin.

 • Infection. Typically, a streptococcal sore throat may precipitate guttate psoriasis.

• Drugs. Beta-blockers, lithium and antimalarials can make psoriasis worse or precipitate it.

   Sunlight. Exposure to sunlight can aggravate psoriasis (in about 10%), although in the majority it has a beneficial effect.

 • Psychological stress. The effects are difficult to assess; most clinicians believe it can exacerbate psoriasis.

CLINICAL FORMS

CLINICAL CLASSIFICATION OF PSORIASIS

NONPUSTULAR                       PUSTULAR

Psoriasis vulgaris                        Palmoplantar pustulosis

 Early onset (type I)                    Pustular psoriasis (annular type)

 Late onset (type II)                    von Zumbusch syndrome (acute febrile

Psoriatic erythroderma               generalized pustular psoriasis)

Type I: early onset (75 %) in females (16 years) and in males (22 years)

Type II: late onset (25 %) in males and females (56 years)

CLINICAL SIGNS

Psoriasis varies in severity from the trivial to the life-threatening. Its appearance and behaviour also range widely from the readily recognizable chronic plaques on the elbows to the acute generalized pustular form. Psoriasis can be confused with other conditions.

Presentation patterns of psoriasis include: plaque, guttate, flexural , localized forms, generalized pustular, nail involvement and erythroderma.

Plaque: Well-defined, disc-shaped plaques involving the elbows, knees, scalp hair margin or sacrum are the classic presentation. The plaques are usually red and covered by waxy white scales which, if detached, may leave bleeding points. Plaques vary in diameter from 2 cm or less to several cm, and are sometimes pruritic.

Guttate: Guttate psoriasis is an acute symmetrical eruption of 'drop-like' lesions usually on the trunk and limbs. This form mostly occurs in adolescents or young adults and may follow a streptococcal throat infection.

Flexural: This variant of psoriasis affects the axillae, sub-mammary areas and natal cleft. Plaques are smooth and often glazed. It is mostly found in the elderly

Localized forms Psoriasis can also present in a number of localized forms:

      Palmoplantar pustulosis is characterized by yellow to browncoloured sterile pustules on the palms or soles . A minority of subjects have classic plaque psoriasis elsewhere. It is most common in middle-aged females, nearly all of whom are cigarette smokers, and it follows a protracted course

     Acrodermatitis ofHallopeau is an uncommon indolent form of psoriasis affecting the digits and nails

      Scalp psoriasis may be the sole manifestation of the disease. It can be confused with dandruff but is generally better demarcated and more thickly scaled.

       Napkin psoriasis is a well-defined psoriasiform eruption in the nappy area of infants, some of whom later develop true psoriasis.

Generalized pustular

Generalized pustular is a rare but serious and even life-threatening form of psoriasis. Sheets of small, sterile yellowish pustules develop on an erythematous background and may rapidly spread. The onset is often acute. The patient is unwell, with fever and malaise, and requires hospital admission

Nail involvement

 Psoriasis affects the matrix or nail bed in up to 50% of cases. Thimble pitting is the commonest change, followed by onycholysis (separation of the distal edge of the nail from the nail bed). An oily or salmon pink discoloration of the nail bed is seen, often adjacent to onycholysis. Subungual hyperkeratosis, with a build-up of keratin beneath the distal nail edge, mostly affects the toe nails. Nail changes are frequently associated with psoriatic arthropathy. Treatment is often difficult.

DIAGNOSIS

A diagnosis of psoriasis is usually based on the appearance of the skin. Skin characteristics typical for psoriasis are scaly, erythematousplaques, papules, or patches of skin that may be painful and itch.^[15] No special blood tests or diagnostic procedures are needed to make the diagnosis.

A diagnosis of psoriasis is usually based on the appearance of the skin. Skin characteristics typical for psoriasis are scaly, erythematousplaques, papules, or patches of skin that may be painful and itch.^[15] No special blood tests or diagnostic procedures are needed to make the diagnosis.

TREATMENT

 Local

Vitamin D analogues ( Calcipotriol and tacalcitol)

Keratolytics and scalp preparations

Topical cortico steroids

Coal tar preparations

Dithranol (Anthralin)

Retinoids

Emollients

Systemic

Methotrexate

Retinoids

Ciclosporin

33. PITYRIASIS ROSEA. ETIOPATHOGENESIS, CLINICAL FEATURES, DIAGNOSTIC, LOCAL AND SYSTEMIC TREATMENT.

Pityriasis rosea is an acute, self-limiting disorder of unknown aetiology characterized by scaly oval papules and plaques which mainly occur on the trunk.

CLINICAL FEATURES

The generalized eruption is preceded in most patients by the appearance of a single lesion, 2-5 cm in diameter, known as a 'herald patch' . Some days later, many smaller plaques appear mainly on the trunk but also on the upper arms and thighs. Individual plaques are oval, pink and have a delicate peripheral 'collarette' of scale. They are distributed parallel to the lines of the ribs, radiating away from the spine. Itching is mild or moderate. The eruption fades spontaneously in. 4-8 weeks. It tends to affect teenagers and young adults. The cause is unknown, but epidemiological evidence of 'clustering' suggests an infective aetiology.

Skin Lesions

 TYPE Herald Patch (80 % of patients) oval, slightly raised plaque 2 to 5 cm, bright red, fine collarette scale at periphery, may be multiple

 Exanthem Fine scaling papules and plaques with typical marginal collarette 

COLOR Dull pink or tawny (exanthem)

SHAPE Oval lesions

ARRANGEMENT Scattered discrete lesions

DISTRIBUTION Characteristic pattern of lesions—the long axes of the lesions follow the lines of cleavage in a “Christmas tree” distribution. Lesions usually confined to trunk and proximal aspects of the arms and legs. Rarely on face.

DIAGNOSTIC

A patient is diagnosed as having pityriasis rosea if:

1.   On at least one occasion or clinical encounter, he / she has all the essential clinical features and at least one of the optional clinical features, and

2.   On all occasions or clinical encounters related to the rash, he / she does not have any of the exclusional clinical features.

The essential clinical features are the following:

1.   Discrete circular or oval lesions,

2.   Scaling on most lesions, and

3.   Peripheral collarette scaling with central clearance on at least two lesions.

The optional clinical features are the following:

1.   Truncal and proximal limb distribution, with less than 10% of lesions distal to mid-upper-arm and mid-thigh,

2.   Orientation of most lesions along skin cleavage lines, and

3.   A herald patch (not necessarily the largest) appearing at least two days before eruption of other lesions, from history of the patient or from clinical observation.

The exclusional clinical features are the following:

1.   Multiple small vesicles at the centre of two or more lesions,

2.   Two or more lesions on palmar or plantar skin surfaces, and

3.   Clinical or serological evidence of secondary syphilis.

TREATMENT

Pruritus may be controlled by UVB phototherapy or natural sunlight exposure if this is begun in the first week of eruption. The protocol is five consecutive exposures, starting with 80 % of the minimum erythema dose and increasing 20 % each exposure.

34. Pemphigus. Etiopathogenesis, clinical forms, clinical signs, diagnostic, local and systemic treatment.

Pemphigus is an uncommon, severe and potentially fatal autoimmune blistering disorder affecting the skin and mucous membranes.

ETIOPATHOGENESIS

Ninety per cent of patients have circulating IgG autoantibodies, detectable in the serum by indirect immunofluorescence, which bind with desmoglein, a desmosomal cadherin involved in epidermal intercellular adhesion. The antibodies, possibly with complement activation and protease

release, result in loss of adhesion and an intraepidermal split. Direct immunofluorescence shows the intercellular deposition of IgG in the suprabasal epidermis. Pemphigus is associated with other organ-specific autoimmune disorders such as myasthenia gravis.

CLINICAL FORMS

Pemphigus Vulgaris

Pemphigus Vegetans

Pemphigus Foliaceus

Pemphigus Erythematosus (PE)

CLINICAL SIGNS

Pemphigus Vulgaris

Primary skin lesion: Bulla: Easily broken, more than  5mm, appears on normal skin, monomorphic

Localisation: trunk, extremities, oral mucosa; just erosion because the blisters are very thin

Complains: severe pain

Age: 40 – 60

Pathogenesis: Fomation of IgG

PathoHistology: Acantholysis +

Symptoms: Nickolyski symptom

Prognosis: Death without treatment

Pemphigus Vegetans

Primary skin lesion: Bulla appears on normal skin with vegetation on the surface of erosion

Localisation: intertiginous regions (axilla, groins)

Complains: severe pain

Age: 40 – 60

Mocous membrane: not affected

Pathogenesis: IgG attached attaches stration spinosum

PathoHistology: Acantholysis +

Symptoms: Nickolyski symptom

Prognosis: Death without treatment

Pemphigus Foliaceus

Primary skin lesion: Bulla, crusts and scales

Localisation: Diffused

Complains: pain

Mocous membrane: not affected

Pathogenesis: same

Pathohistology: same

Symptoms: same

Prognosis: Death without treatment

Primary skin lesion: Bulla

Localisation: Seborrehic region, face, chest, back

Complains: same

Age: same

Pathogenesis: same

Pathohistology: same

Symptoms: same

Prognosis: Death without treatment

DIAGNOSIS

1.     Microscopic investigation

2.     Nickolyski symptom

3.     Elisa

Can be a difficult, subtle problem if only mouth lesions are present. Biopsy of the skin and mucous membrane, direct immunofluorescence, and demonstration of circulating autoantibodies confirm a high index of suspicion.

TREATMENT

Systemic Corticosteroids: Prenisolone 90 – 120 mg per day

Morning 60 mg

Afternoon 40mg

Evening 20mg

Concomitant Immunosuppressive Therapy Immunosuppressive agents are given concomitantly for their corticosteroid-saving effect:

    Azathioprine, 2.0 to 3.0 mg/kg of body weight until complete clearing; tapering of dose to 1.0 mg/kg. Azathioprine alone is continued even after cessation of corticosteroid treatment and may have to be continued for many months. Clinical freedom from disease and a negative pemphigus antibody titer for at least 3 months permit cessation of therapy.

    Methotrexate, either PO or IM at doses of 25 to 35 mg per week. Dose adjustments are made as with azathioprine.               Cyclophosphamide, 100 to 200 mg daily, with reduction to maintenance doses of 50 to 100 mg/day. Alternatively, cyclophosphamide “bolus” therapy with 1000 mg IV once a week or every 2 weeks in the initial phases, followed by 50 to 100 mg/day PO as maintenance.

   Gold therapy, for milder cases. After an initial test dose of 10 mg IM, 25 to 50 mg of gold sodium thiomalate is given IM at weekly intervals to a maximum cumulative dose of 1 g.

35. Pemphigoid. Etiopathogenesis, clinical forms, clinical signs, diagnostic, local and systemic treatment.

Pemphigoid is an autoimmune disorder presenting as a chronic bullous eruption mostly in patients over 60 years of age.

ETIOPATHOGENESIS

IgG autoantibodies to bullous pemphigoid antigens in the hemidesmosomes at the basement membrane zone bind complement which induces inflammation and protease release, leading to subepidermal bulla formation. The IgG and C3 are detected by direct immunofluorescence. Indirect

methods demonstrate circulating autoantibodies in 70% of cases.

CLINICAL FORMS

Bullous pemphigoid

Cicatritial pemphigoid

CLINICAL SIGNS

Bullous pemphigoid

Primary skin lesion: Bulla - hard

Localisation: extremities; flexural regions, mucous membrane maybe affected

Complains: Pruritis in place of bullae

Age: 70>

Mocous membrane: affected

Pathogenesis: IgG attached attaches stration spinosum

PathoHistology: Acantholysis absent

Symptoms: Nickolyski symptom negative

Prognosis: Not fatal

Cicatritial pemphigoid

Primary skin lesion: Bulla not hard with scar formation

Localisation: usually of mucous membrane of mouth, eyes, nose, throat, genitals

Complains: loss of function of affected system

Age: 60>

Mocous membrane: affected

Pathogenesis: IgG attached attaches stration spinosum

PathoHistology: Acantholysis absent

Symptoms: Nickolyski symptom negative

Prognosis: Not fatal

DIAGNOSTIC

Clinical, confirmed by histopathology and immunopathology

Microscopy

Elisa

TREATMENT

Systemic prednisone with starting doses of 50 to 100 mg to clear, either alone or combined with azathioprine 150 mg daily for remission induction and 50 to 100 mg for maintenance; in milder cases, sulfones (dapsone), 100 to 150 mg/day. In very mild cases and for local recurrences, topical corticosteroid therapy may have a beneficial effect. Tetracycline 6 nicotinamide has been reported to be effective in some cases.

36. Dermatitis Herpetiformis. Etiopathogenesis, clinical features, diagnostic, local and systemic treatment.

Dermatitis herpetiformis (DH) is a chronic, recurrent, intensely pruritic eruption on

extensor surfaces occurring often in symmetric groups and comprising three types

of lesions: tiny vesicles, papules, and urticarial wheals. . Jejunal villus atrophy is an associated finding in most cases.

ETIOPATHOGENESIS

DH is characterized by the finding of granular IgA at the dermal papillae on immunofluorescence, and by the response of the skin lesions (and the villus atrophy seen in over 75% of patients) to a gluten-free diet. Despite this, the cause of the eruption - and its relationship to the undoubted gluten sensitivity of both the gut and the skin - remains unclear. It is doubtful whether the IgA induces the itch, as it is present in asymptomatic patients.

CLINICAL FEATURES

Duration of Lesions Days, weeks

Skin Symptoms Pruritus, intense, episodic; burning or stinging of the skin; rarely, pruritus may not occur.

Local symptoms often precede the appearance of skin lesions by 8 to 12 hours.

Exacerbating Factors Ingestion of iodides and overload of gluten. Provocation with iodides administered orally has been used diagnostically in the past. The availability of immunopathologic techniques and the detection of IgA deposits in the skin have made such provocation tests obsolete.

Systems Review Laboratory evidence of small-bowel malabsorption is detected in 10 % to 20 %. Gluten-sensitive enteropathy occurs in nearly all patients and is demonstrated by small-bowel biopsy. There are usually no systemic symptoms.

Skin Lesions 

TYPES Erythematous papule. Tiny firm-topped vesicle, sometimes hemorrhagic; occasionally bullae. Urticaria-like wheal. Scratching results in excoriations, crusts. Postinflammatory hyper- and hypopigmentation at sites of healed lesions.

COLOR Red

ARRANGEMENT In groups (hence the name herpetiformis)

DISTRIBUTION Strikingly symmetric.

SITES OF PREDILECTION Extensor areas—elbows, knees. Buttocks, scapular, sacral area. Scalp, face, and hairline

DIAGNOSIS

Often difficult. Biopsy of early lesions is usually pathognomonic, but immunofluorescence detecting IgA deposits in normal-appearing or perilesional skin is the best confirming evidence.

TREATMENT

Systemic Therapy

DAPSONE 100 to 200 mg daily with gradual reduction to 25 to 50 mg

SULFAPYRIDINE 1.0 to 1.5 g daily, with plenty of fluids, if dapsone contraindicated or not tolerated

 Diet: A gluten-free diet may completely suppress the disease or allow reduction of the dosage of dapsone or sulfapyridine, but response is slow.

37. Lupus Erythematosus. Etiopathogenesis, clinical forms, clinical signs, diagnostic, local and systemic treatment.

This serious multisystem disease involves connective tissue and blood vessels; the

clinical manifestations include fever (90 %), skin lesions (85 %), arthritis, and renal,

cardiac, and pulmonary disease.

ETIOPATHOGENESIS

Autoantibodies to nuclear, nucleolar and cytoplasmic antigens are found in

LE. Over 90% of systemic LE patients have circulating antinuclear antibodies

as compared to less than 25% of those with discoid LE. Subjects with systemic

LE have impaired T-cell immunity. UV radiation often brings out the eruption,

perhaps by generating nuclear products in the skin. Systemic LE is

associated with HLA phenotypes B8 and DR3, and certain drugs may

trigger it.

CLINICAL FORMS

Systemic LE

Skin signs are found in 75%. The facial butterfly eruption is well known, but photosensitivity, discoid lesions, diffuse alopecia and vasculitis also occur. Multisystem involvement with serological or haematological abnormalities must be demonstrated

to diagnose systemic LE. The female: male ratio is 8:1.

Discoid LE

One or more round or oval plaques appear on the face, scalp or hands. The lesions are welldemarcated, red, atrophic, scaly and show keratin plugs in dilated follicles.

Scarring leaves alopecia on the scalp and hypopigmentation in those with a pigmented skin. Remission occurs in 40%. Internal involvement is not a feature, and only 5% develop systemic

LE. Women outnumber men by 2:1.

Other forms

Subacute cutaneous LE is characterized by widespread symmetrical scaly plaques on sun-exposed sites of the face and forearms. Internal disease may occur, and anti-Ro antibodies are found.

Neonatal LE is due to the placental transfer of anti-Ro antibodies from affected mothers to their neonates, who develop an annular atrophic eruption, sometimes with heart block.

CLINICAL SIGNS

Skin Lesions

TYPES

Erythematous, confluent, macular butterfly eruption (face) with fine scaling; erosions and crusts

Erythematous, discrete, papular or urticarial lesions (face, dorsa of hands, and arms)

Bullae, often hemorrhagic (acute flares)

Papules and discoid plaques as in CCLE (face and arms)

“Palpable” purpura (vasculitis)

Urticarial lesions with purpura (“urticarial vasculitis”)

COLOR Bright red

SHAPE Round or oval

ARRANGEMENT Diffuse involvement of the face in light-exposed areas. Scattered

discrete lesions (face, forearms, and dorsa of hands).

DISTRIBUTION Localized or generalized

SITES OF PREDILECTION Face (80 %); scalp (discoid lesions); presternal, shoulders;

dorsa of the forearms, hands, fingers, fingertips (vasculitis); palms; periungual

telangiectases and palmar erythema are also seen.

Hair Discoid lesions associated with patchy alopecia. Diffuse alopecia

Mucous Membranes Ulcers arising in purpuric necrotic lesions on palate (80 %),

buccal mucosa, or gums

Extracutaneous Multisystem Involvement Arthralgia or arthritis (15 %), renal

disease (50 %), pericarditis (20 %), pneumonitis (20 %), gastrointestinal (due to

arteritis and sterile peritonitis), hepatomegaly (30 %), myopathy (30 %), splenomegaly

(20 %), lymphadenopathy (50 %), peripheral neuropathy (14 %), CNS disease

(10 %), seizures or organic brain disease (14 %)

DIAGNOSTIC

The criteria for diagnosing the condition include

at least four of the features

Criteria for diagnosing systemic lupus erythematosus

• Malar rash

• Serositis

• Discoid plaques

• Neurological involvement

• Photosensitivity

• Haematological changes

• Arthritis

• Immunological changes

• Mouth ulcers

• Antinuclear antibodies

• Renal changes

LOCAL AND SYSTEMIC TREATMENT.

Discoid LE usually responds to potent or very potent topical steroids which,

in this instance, can be applied to the face. Sunblock creams are helpful.

Widespread disease may need systemic therapy with hydroxychloroquine: the

small risk of retinopathy demands regular tests of visual acuity.

The treatment of systemic LE depends on the type of involvement. Sunscreens

reduce photosensitivity, but if there is internal disease, systemic steroids are

required, often with immunosuppressive agents.

38. Scleroderma. Etiopathogenesis, clinical features, diagnostic, local and systemic treatment.

Scleroderma is a multisystem disorder characterized by inflammatory, vascular, and

sclerotic changes of the skin and a variety of internal organs, especially the lungs,

heart, and GI tract.

Synonyms: Progressive systemic sclerosis, systemic sclerosis, systemic scleroderma

ETIOPATHOGENESIS

Etiology Unknown

Age Onset 30 to 50 years

Sex Female : males 4 : 1

Pathogenesis unknown. Primary event might be endothelial cell injury in blood

vessels, the cause of which is unknown. Early in course, target organ edema occurs,

followed by fibrosis; cutaneous capillaries are reduced in number; remainder dilate

and proliferate, becoming visible telangiectasia. Fibrosis due to overproduction of

collagen by fibroblasts.

CLINICAL FEATURES

Physical Examination

Skin, Hair, and Nail Findings

TYPES OF LESIONS Hands/Feet Early: Raynaud’s phenomenon with

triphasic color changes, i.e., pallor, cyanosis, rubor.

Nonpitting edema of hands/feet. Painful ulcerations at fingertips,

 knuckles; heal with pitted scars. Late: sclerodactyly with

tapering fingers, waxy, shiny atrophic skin, which is tightly bound

down; flexion contractures; bony resorption results in loss of distal

phalanges

Face Early: periorbital edema. Late: edema and fibrosis result in loss of

normal facial lines, masklike, thinning of lips, microstomia,

radial perioral furrowing, small sharp nose.

Ulceration Secondary to vascular occlusion, acrally

Cutaneous Calcification Occurs over bony prominences or any sclerodermatous

site; may ulcerate and extrude white paste.

CREST Syndrome Matlike telangiectasia, especially on

face, neck, upper trunk, hands; also, lips, oral mucous membranes, GI tract

COLOR Hyperpigmentation, generalized. In areas of sclerosis, postinflammatory

hyper- and hypopigmentation

PALPATION Early: skin feels indurated, stiff. Late: tense, smooth, hardened, bound

down. Leathery crepitation over joints, especially knees.

DISTRIBUTION OF LESIONS Early: fingers, hands. Late: upper extremities, trunk, face,

lower extremities

HAIR AND NAILS Thinning/complete loss of hair on distal extremities. Loss of sweat

glands with anhidrosis. Periungual telangiectasia with giant sausage-shaped capillary

loops. Nails grow clawlike over shortened distal phalanges

MUCOUS MEMBRANES Sclerosis of sublingual ligament; uncommonly, painful induration

of gums, tongue

General Examination

ESOPHAGUS Dysphagia, diminished peristalsis, reflux esophagitis

GASTROINTESTINAL SYSTEM Small intestine involvement may produce constipation,

diarrhea, bloating, and malabsorption.

LUNG Restricted movement of chest wall due to pulmonary fibrosis and alveolitis.

Reduction of pulmonary function

HEART Cardiac conduction defects, heart failure, pericarditis

KIDNEY Renal involvement occurs in 45 %. Slowly progressive uremia, malignant

hypertension.

MUSCULOSKELETAL SYSTEM Carpal tunnel syndrome. Muscle weakness.

DIAGNOSIS

Clinical findings confirmed by dermatopathology.

Laboratory and Special Examinations

Dermatopathology Early: mild cellular infiltrate around dermal blood vessels,

eccrine coils, subcutaneous tissue. Late: epidermis shows disappearance of rete

ridges, increased eosinophilia, broadening and homogenization of collagen bundles,

obliteration and decrease of interbundle spaces, thickening of dermis with replacement

of upper or total subcutaneous fat by hyalinized collagen. Paucity of blood

vessels, thickening/hyalinization of vessel walls, narrowing of lumen; dermal

appendages atrophied; sweat glands are situated in upper dermis; calcium in

sclerotic, homogeneous collagen of subcutaneous tissue.

Autoantibodies Patients with dSSc have circulating autoantibodies by ANA

testing. Autoantibodies react with centromere proteins or DNA topoisomerase I;

fewer patients have antinucleolar antibodies. Anticentromeric autoantibodies occur

in 21 % of dSSc and 71 % of CREST, DNA topoisomerase I (Scl-70) antibodies in

33 % of dSSc and 18 % of CREST.

Cardiac Studies

Pulmonary Studies Diffusion studies: reduced O2 transport

TREATMENT

Systemic corticosteroids may be of benefit for limited periods early in the disease.

All other systemic treatments (EDTA, aminocaproic acid, D-penicillamine, paraaminobenzoate,

colchicine, immunosuppressive drugs) have not been shown to be

of lasting benefit. Presently, interferon-gamma is being tested clinically, and so is

photopheresis.

Topical Steroid for local treatment.

There is no cure for scleroderma, although relief of symptoms is often achieved. These include

·         Raynaud's phenomenon with vasodilators such as calcium channel blockers, alpha blockers, serotonin receptor antagonists,angiotensin II receptor inhibitors, statins, local nitrates or iloprost

·         Digital ulcers with phosphodiesterase 5 inhibitors (e.g., sildenafil) or iloprost

·         Prevention of new digital ulcers with bosentan

·         Malnutrition, secondary to intestinal flora overgrowth with tetracycline antibiotics like tetracycline

·         Alveolitis with cyclophosphamide, azathioprine with or without corticosteroids

·         Pulmonary arterial hypertension with endothelin receptor antagonists, phosphodiesterase 5 inhibitors and prostanoids

·         Gastrooesophageal reflux disease with antacids or prokinetics

·         Kidney crises with angiotensin converting enzyme inhibitors and angiotensin II receptor antagonists

39. LEPROSY. ETIOPATHOGENESIS, CLINICAL FORMS, CLINICAL SIGNS, DIAGNOSTIC, TREATMENT.

Leprosy is a chronic granulomatous disease caused by Mycobacterium leprae and

principally acquired during childhood or young adulthood. The skin, mucous

membrane of the upper respiratory tract, and peripheral nerves are the major sites

of involvement in all forms of leprosy.

Synonym: Hansen’s disease

ETIOPATHOGENESIS

Etiology M. leprae is a slender, straight, or slightly curved, acid-fast rod, about 3.0

by 0.5 mm. The organism cannot be cultured in vitro.

Pathophysiology

The clinical spectrum of leprosy depends exclusively on variable limitations in the

host’s capability to develop effective cell-mediated immunity (CMI) to M. leprae.

The organism is capable of invading and multiplying within peripheral nerves and

infecting and surviving within endothelial and phagocytic cells in many organs. The

clinical spectrum of disease depends on variable limitations in the host’s capacity to

develop effective cell-mediated immune responses to M. leprae.

Transmission Mode of transmission of M. leprae is uncertain; however, human-tohuman

transmission is the norm. The main source of dissemination is individuals

with multibacillary-type infection, shedding several millions of bacilli per day from

nasal and upper respiratory tract secretions. Portals of entry of M. leprae are poorly

understood but include ingestion of food or drink, inoculation into or through skin

(bites, scratches, small wounds, tattoos), or inhalation into nasal passages or lungs.

CLINICAL FORMS

Clinicopathologic Classification of Leprosy

(Based on clinical, immunologic, and bacteriologic findings.)

Tuberculoid (TL) Localized skin involvement and/or peripheral nerve involvement;

few organisms are present in the skin biopsies.

Lepromatous (LL) Generalized involvement including skin, upper respiratory

mucous membrane, the reticuloendothelial system, adrenal glands, and testes; many

bacilli are present in tissue.

Borderline (or “Dimorphic”) (BL) Has features of both tuberculoid and lepromatous

leprosy. Usually many bacilli present, varied skin lesions: macules, plaques;

progresses to tuberculoid or regresses to lepromatous.

Indeterminate and Transitional Forms

CLINICAL SIGNS

Physical Examination

Tuberculoid Leprosy

TYPE OF LESION A few well-defined hypopigmented anesthetic macules

 with raised edges and varying in size from a few

millimeters to very large lesions covering the entire trunk

COLOR OF LESION Erythematous or purple border and hypopigmented center

BORDER OF LESION Sharply defined, raised

SHAPE OF LESIONS Often annular

DISTRIBUTION OF LESIONS Any site including the face

NERVE INVOLVEMENT May be a thickened nerve on the edge of the lesion; large

peripheral nerve enlargement frequent (ulnar)

Lepromatous Leprosy

TYPES OF LESIONS Small erythematous or hypopigmented macules that

are anesthetic; later papules, plaques, nodules, and

diffuse thickening of the skin, with loss of hair (eyebrows and

eyelashes). Facies leonina (lion’s face) due to thickening, nodules and plaques distort

normal facial features.

COLOR OF LESIONS Normal skin color or erythematous or slightly hypopigmented

DISTRIBUTION OF LESIONS Bilaterally symmetric involving earlobes, face, arms, and

buttocks, or less frequently the trunk and lower extremities

MUCOUS MEMBRANES Tongue: nodules, plaques, or fissures

Borderline Leprosy Lesions are intermediate between tuberculoid and lepromatous

and comprised of macules, papules, and plaques. Anesthesia and decreased

sweating are prominent in the lesions.

Reactional Phenomenon

LEPRA TYPE 1 REACTIONS Skin lesions become acutely inflamed associated with

edema and pain; may ulcerate. Edema most severe on face, hands, and feet.

LEPRA TYPE 2 REACTIONS (ENL) Present as painful red skin nodules, arising superficially

and deeply; lesions form abscesses or ulcerate. Lesions occur most commonly

on face and extensor limbs.

LUCIO’S REACTION Presents as irregularly shaped erythematous plaques; lesions

may resolve spontaneously or undergo necrosis with ulceration.

DIAGNOSIS

Made if one or more of the cardinal findings are detected: skin lesions characteristic

of leprosy with diminished or loss of sensation, enlarged peripheral nerves, finding

of M. leprae in skin or, less commonly, other sites.

Laboratory and Special Examinations

Slit-Skin Smears A small skin incision is made; the site is then scraped in order to

obtain tissue fluid, which is examined following Ziehl-Neelsen staining. Specimens

are usually obtained from both earlobes and two other active lesions.

Culture M. leprae has not been cultured in vitro; however, it does grow when

inoculated into the mouse foot pad. Routine bacterial cultures to rule out secondary

infection.

PCR M. leprae DNA detected by this technique makes the diagnosis of early

paucibacillary leprosy and identifies M. leprae following therapy.

Dermatopathology TL shows epithelioid cell granulomas forming around dermal

nerves; AFBs are sparse or absent. LL shows an extensive cellular infiltrate separated

from the epidermis by a narrow zone of normal collagen. Skin appendages are

destroyed. Macrophages are filled with M. leprae, having abundant foamy or vacuolated

cytoplasm (lepra cells or Virchow cells).

TREATMENT

The two most widely used drugs are dapsone and rifampin, which are generally

available. Clofazimine and thalidomide are available through the National Hansen’s

Disease Center in Carville, Louisiana.

General principles of management include: eradicate infection with antilepromatous

therapy, prevent and treat reactions, reduce the risk of nerve damage, educate patient

to deal with neuropathy and anesthesia, treat complications of nerve damage,

rehabilitate patient into society.

40. Cutaneous Tuberculosis. Etiopathogenesis, clinical forms, clinical signs, diagnostic, treatment.

Cutaneous tuberculosis (CTb) is highly variable in its clinical presentation, depending on the immunologic status of the patient and the route of inoculation of mycobacteria into the skin.

CLINICAL FORMS

Classification of Cutaneous Tuberculosis

Exogenous Infection

Primary inoculation tuberculosis (PITb): via percutaneous inoculation occurs at the inoculated site in a nonimmune host.

Tuberculosis verrucosa cutis (TbVC): via percutaneous inoculation occurs at the inoculated site in an individual with prior tuberculosis infection.

Endogenous Spread

Lupus vulgaris (LV)

Scrofuloderma (SD)

Metastatic tuberculosis abscess (MTbA)

Acute miliary tuberculosis (AMTb)

Orificial tuberculosis (OTb)

Tuberculosis due to BCG Immunization

CLINICAL SIGNS

Primary Inoculation Tuberculosis

TYPES OF LESIONS Initially, papule occurs at the inoculation site 2 to 4 weeks after the wound. Lesion enlarges to a painless ulcer, i.e., a tuberculous chancre (up to 5 cm), with shallow granular base and multiple tiny abscesses or, alternatively, may be covered by thick crust. Undermined margins; older ulcers become indurated with thick crusts. Deeper inoculation results in subcutaneous abscess. Intraoral inoculation results in ulcers on gingiva or palate. Regional lymphadenopathy occurs within 3 to 8 weeks.

COLOR OF LESIONS Ulcer margins reddish blue

DISTRIBUTION OF LESIONS Most common on exposed skin at sites of minor injuries. Oral lesions occur following ingestion of bovine bacilli in nonpasteurized milk; in the past, lesions in male babies have occurred on the penis after ritual circumcision

Tuberculosis Verrucosa Cutis

TYPES OF LESIONS Initial papule with violaceous halo. Evolves to hyperkeratotic, warty, firm plaque (Figure 24-24). Clefts and fissures occur from which pus and keratinous material can be expressed. Border often irregular. Lesions are usually single, but multiple lesions occur. No lymphadenopathy.

COLOR OF LESIONS Base brownish red to purplish

DISTRIBUTION OF LESIONS Most commonly on dorsolateral hands and fingers. In children, lower extremities, knees.

Lupus Vulgaris

TYPES OF LESIONS Initial flat papule is ill-defined and soft and evolves into well-defined, irregular plaque (Figure 24-25). The consistency is characteristically soft; if the lesion is probed, the instrument breaks through the overlying epidermis. Surface is initially smooth or slightly scaly but may become hyperkeratotic. Hypertrophic forms result in soft tumorous nodules. Ulcerative forms present as punched-out, often serpiginous ulcers surrounded by soft, brownish infiltrate. Involvement of underlying cartilage but not bone results in its destruction (ears, nose). Scarring is prominent, and characteristically, new brownish infiltrates occur within the atrophic scars. COLOR OF LESIONS Reddish brown. Diascopy (i.e., the use of a glass slide pressed against the skin) reveals an “apple jelly” (i.e., yellowish brown) color. DISTRIBUTION OF LESIONS Usually solitary, but several sites may occur. Most lesions on the head and neck, most often on nose and ears or scalp. Lesions on trunk and extremities rare. Disseminated lesions after severe viral infection (measles) (lupus postexanthematicus).

DIAGNOSIS

Diagnosis Clinical, histologic findings, confirmed by isolation of M. tuberculosis on culture

Laboratory and Special Examinations

Dermatopathology PITb: initially nonspecific inflammation; after 3 to 6 weeks, epithelioid cells, Langhans giant cells, lymphocytes, caseation necrosis. AMTb: nonspecific inflammation and vasculitis. All other forms of CTb show more or less typical tuberculous histopathology; TbVC is characterized by massive pseudoepitheliomatous hyperplasia of epidermis and abscesses. Mycobacteria are found in PITb, SD, AMTb, MTbA, OTb, but only with difficulty or not at all in LV and TbVC.

Culture Yields mycobacteria also from lesions of LV and TbVC

PCR Can be used to identify T. tuberculosis DNA in tissue specimens

Skin Testing PITb: Patient converts from intradermal skin test negative to positive during the first weeks of the infection. AMTb: usually negative. SD, MTbA, and OTb: may be negative or positive depending on state of immunity. LV and TbVC: positive.

TREATMENT

Only PITb and TbVC limited to the skin. All other patterns of CTb are associated with systemic infection that has disseminated secondarily to the skin. As such, therapy should be aimed at achieving a cure, avoiding relapse, and preventing emergence of drug-resistant mutants.

 Antituberculous Therapy

Prolonged antituberculous therapy with at least two drugs is indicated for all cases of CTb except for TbVC that can be excised. Isoniazid and rifampin, supplemented with ethambutol, streptomycin or pyrizinamid in the initial phases. Isoniazid and rifampin for at least 9 months; can be shortened to 6 months if four drugs are given during the first 2 months.