天疱疮 （pemphigus）课件

Pemphigus and pemphigoid (pemphigus) definition：autoimmune skin and mucous membrane vesiculobullous disease is characterized with acantholysis,bullae formation in the intraepidermal areas caused by intercellular antibodies deposited in stratum spinosum classification：four types two species vulgaris type pemphigus vulgaris （bullae at underlayer of acanthocytes） pemphigus vegetans foliaceus type pemphigus foliaceus （bullae at superficial layer of acanthocytes） pemphigus erythematosus pathogenesy Evidence of autoimmune disease： lIg deposited between acanthocytes of skin lesion lPemphigus antibodies are present in serum antibody titer related with pathogenetic condition lPlasmapheresis removing pemphigus antibodies is useful in a short term lpemphegus antibodies transfer fetus across placenta lHigh titer pemphigus antibodies repeatedly intradermal injection induced acantholysis Clinical features Incidence of pemphigus per year0.53.2/100,000 1. Pemphigus vulgaris lUsually occurs in middle-age（age of 60） lBlister and erosion usually appear on oral mucosa lthin-walled,flaccid bullae or enlarged erosion suface lNikolsky sign positive lLesions appear in the scalp,face,chest and back 2. pemphigus vegetans Hallopeau Nenmann The lesions limited in axillae、 groin 、under breast、 perineum, etc. 3. pemphigus foliaceus lLesions are at upper layer of acanthocytes lThin-walled bullae or porous thick crusts lLesions aggravate after exposed to sunlight pemphigus erythematosus lesions appear most commonly in the scalp、face、superior part of trunk Erythema and flaccid bulla Nikolsky sign （） Immunologic test ldirect immunofluorecence （DIF） ： in acantholytic areas IgG、 IgM、IgAor C3 depostion Indirect immunofluorecence （IIF） Circulating intercellular antibodies can be demonstrated in 8090 of patients serum with pemphigus vulgaris Mainly IgG Antibody titer often parallel disease activity histopathology pemphigus vulgaris lBlster intraepidermal lAcantholysis in bullae （pemphigus cell） Diagnosis and antidiastole Pemphigus vulgaris（1） lUsually occurs in the elderly（around age of 60） lBlister and erosion usually appear on oral mucosa lthin-walled,flaccid bullae or enlarged erosion suface lNikolsky sign positive lLesions appear in the scalp,face,chest and back Diagnosis and antidiastole Pemphigus vulgaris（2） lDIF shows Ig deposited intercellular lIIF shows pemphigus antibodies lPathology shows blister intraepidermal，acantholysis treatment 1. General treatment lSurpportive therapy、vitamin and protein supplement lTransfusion and eleltrolyte balance lAttending raw surfaces，prevent infection lCaution the complication 2. corticosteroid choice drug，early、full dose 、maintenance 3. Immunosuppresant azathioprine or cytoxan 4.dapsone（D.D.S) 5.wilfordii preparation 6. plasmaphoresis 7. Large dose IVIg stosstherapy 0.4g / kg / d 35d pemphigoid definition An autoimmunity dermatosis mainly occurs in aged people ， characterized with blister formation intraepidermal classification l Bullous pemphigoid （ BP） lLocalized pemphigoid l小疱型类天疱疮 l结节痒疹型天疱疮 lPleomorphous pemphigoid lCicatricial pemphigoid Pemphegoid clinical features （BP） lusually occurs in the elderly（over age of 70） lthic-walled tense bullae or blister lMaily apear in axillae、groin lHistopathology shows bullae subepidermal lDIF shows C3 and/or IgG linear deposited in the basement of the membrane zone treatment 1. General treatment lHigh protein ingest lPrevent skin secondary infection lEliminate malignant tumor 2. corticosteroid choice drug，e