PEMFIGUS VULGARIS

Yuli Kurniawati Department of Dermatovenereology Faculty of Medicine Sriwijaya University/ General Hospital Moch Hoesin Palembang

Skin

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hemodesmosome and epidermal basement membrane are targeted in autoimun skin blistering diseases. Hemidesmosome and the epidermal basement membrane are the providers of epidermal and epidermal cohesion • Protein components of desmosomes. .Introduction • Desmosome.

PEMPHIGUS VULGARIS • Group of autoimmune diseases of skin and mucous membranes • Characterized histologically by epidermal blisters due to acantolysis • Prevalence men and women nearly equal • The mean age of onset 40-60years .

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ASBOE HANSEN sign (+) . but may develop on erythematous skin. scalp. • The blisters are fragille • NIKOLSKY SIGN (+).Clinical manifestations • Predilection: intertriginous area. face • Skin lessions are rare itch but painful • Primary lesion : flaccid blister usually the blister arise on normal appearing skin.

Skin lessions .

MUCOUS MEMBRANES • Painful mucous membrane erosions • The erosions may spread to pharinx. larynx  hoarseness .

.Histopathology : • Suprabasal blisters with acantholyis just above the basal cell layer.

Histopathology… • Basal cells stay attached to the basement membrane but may loose contact  “row of tombstones” .

transmembrane desmosomal adhesion molecules .Immunopathology • Direct immunofluoresence : Ig G on the keratinocyte cell surface • Indirect immunofluoresence : Ig G in serum patient’s • Autoantigens : desmosomes.

Differential diagnosis .

Therapy • Localized pemphigus : topical glucocorticoid • Generalized phemphigus : systemic glucocorticoid  long time steroid with tappering dose • Dose : Prednisone 1 mg/kg/day • Th/ adjuvant steroid sparing agents: azathioprine. cyclosphosphamide . mycophenolate mofetil.

Prognosis • Before the advent of glucocorticoid therapy  almost fatal in elderly patients with concurrent medical problems • With glucocorticoid and immunosuppressive therapy mortality more less .

Bolluos Pemphigoid Epidemiology : • onset older than 60years old • Rarely in children • The incidence 7per million per year in France and German .

Etiology and pathogenesis • Early blister formation : lamina lucida • Lost of anchoring filaments and hemidesmosomes • Pemphigoid antigens : BPAG1. BGAG2 .

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inner or anterior thighs. early lessions  Urticarial lession • Resolution  hyperpigmentation • Mucous membrane : 10-35 % . flexor forearm • Bullae usually are filled with clear fluid  hemorragic • Usually Pruritus . tense blister arising on normal skin or erythematous base • Predilection : lower abdomen.Clinical Manifestations • Skin lessions : large.

Skin lession .

Differential diagnosis .

Laboratory • Elevated total serum Ig G • Eosinophilia .

eosinophils. with superficial dermal infiltrate lymphocytes.Histopathology • Sub epidermal blister without epidermal necrosis. . histiocytes.

Immunopatology • Direct Immunofluorescence : C3 and IgG deposited in linear pattern at the epidermal basement membrane • Indirect Immonofluarescence : anti basement membrane Ig G in serum • Autoantigens : protein in the keratinocyte hemidesmosome. basal cell membrane adhesion structure .

Immunofluorescence .

Therapy Corticosteroids : • High potency topical • Prednisone Immonosuppressive agents : azathioprine. Dapsone. mycophenolate mofetil Modulators of antibody levels: • Intravenous gammaglobulin • Plasmapheris Other : tetracycline. Topical tacrolimus . Nicotinamide.

Prognosis • Mortality : 6-40 % in 1 year .

000 persons • Onset any age from childhood • Associated Gluten sensitive enteropathy .Duhring Disease Sinonim : Dermatitis Herpetiformis (DH) Epidemiology : • various Caucasian 10-39/100.

Etiology and pathogenesis • Dieterich et al 1999 : identified antibody to tissue transglutaminases ( Tgases) in sera from DH patients • Gluten a protein in wheat. rye  critical role in pathogenesis DH • Ig A skin deposits play in role in patophysiology of blister formation  (?) . barley.

most commonly vesicles. burning and itching . urticarial like plaque. shoulders. buttock. knee.Clinical manifestation • Primary lession : erythematous papulae. sacral area • Stinging. bullae are infrequently • Predilection : usual symetric distribution on elbow.

Skin lession .

eosinophil. separation of the papillary tips from overlying epidermis .Histopatology • Early lession : dermal papillary colections of neutrophils ( microabses).

Differential diagnosis • • • • • • Eczema Atopic dermatitis Papular urticaria Neurotic excoriation Bullous Pemphigoid Linnear Ig A dermatosis .

Immunopathology • Granular Ig A deposit in normal appearing skin .

Therapy • Dapsone 100-150 mg/day • Gluten Free diet • Therapy Symtomatic .

Refences • Fitzpatrick ‘s dermatology in general medicine • Andrew’s disease of the skin Clinical Dermatology .