Pemphigus and pemphigoid

1. Pemphigus and pemphigoid

2. (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

3. 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

4. pathogenesy Evidence of autoimmune disease： • Ig deposited between acanthocytes of skin lesion • Pemphigus antibodies are present in serum antibody titer related with pathogenetic condition • Plasmapheresis removing pemphigus antibodies is useful in a short term • pemphegus antibodies transfer fetus across placenta • High titer pemphigus antibodies repeatedly intradermal injection induced acantholysis

6. Desmoglein 3 desmoglein 3 桥粒核心糖蛋白 3 1

8. Clinical features Incidence of pemphigus per year0.5~3.2/100,000 1. Pemphigus vulgaris • Usually occurs in middle-age（age of 60） • Blister and erosion usually appear on oral mucosa • thin-walled,flaccid bullae or enlarged erosion suface • Nikolsky sign positive • Lesions appear in the scalp,face,chest and back

11. 2. pemphigus vegetans Hallopeau Nenmann The lesions limited in axillae、groin 、under breast、perineum, etc.

13. 3. pemphigus foliaceus • Lesions are at upper layer of acanthocytes • Thin-walled bullae or porous thick crusts • Lesions aggravate after exposed to sunlight

15. pemphigus erythematosus lesions appear most commonly in the scalp、face、superior part of trunk Erythema and flaccid bulla Nikolsky sign （＋）

16. Immunologic test • direct immunofluorecence（DIF） ： in acantholytic areas IgG、 IgM、IgAor C3 depostion

18. Indirect immunofluorecence （IIF） Circulating intercellular antibodies can be demonstrated in 80～90％ of patients’ serum with pemphigus vulgaris Mainly IgG Antibody titer often parallel disease activity

19. histopathology pemphigus vulgaris • Blster intraepidermal • Acantholysis in bullae （pemphigus cell）

21. Diagnosis and antidiastole Pemphigus vulgaris（1） • Usually occurs in the elderly（around age of 60） • Blister and erosion usually appear on oral mucosa • thin-walled,flaccid bullae or enlarged erosion suface • Nikolsky sign positive • Lesions appear in the scalp,face,chest and back

22. Diagnosis and antidiastole Pemphigus vulgaris（2） • DIF shows Ig deposited intercellular • IIF shows pemphigus antibodies • Pathology shows blister intraepidermal，acantholysis

23. treatment 1. General treatment • Surpportive therapy、vitamin and protein supplement • Transfusion and eleltrolyte balance • Attending raw surfaces，prevent infection • Caution the complication

24. 2. corticosteroid choice drug，early、full dose、maintenance 3. Immunosuppresant azathioprine or cytoxan 4.dapsone（D.D.S) 5.wilfordii preparation

25. 6. plasmaphoresis 7. Large dose IVIg stosstherapy 0.4g / kg / d ×3～5d

26. pemphigoid definition An autoimmunity dermatosis mainly occurs in aged people ，characterized with blister formation intraepidermal

27. classification • Bullous pemphigoid（BP） • Localized pemphigoid • 小疱型类天疱疮 • 结节痒疹型天疱疮 • Pleomorphous pemphigoid • Cicatricial pemphigoid

28. Pemphegoid clinical features（BP） • usually occurs in the elderly（over age of 70） • thic-walled tense bullae or blister • Maily apear in axillae、groin • Histopathology shows bullae subepidermal • DIF shows C3 and/or IgG linear deposited in the basement of the membrane zone

32. treatment 1. General treatment • High protein ingest • Prevent skin secondary infection • Eliminate malignant tumor

33. 2. corticosteroid choice drug，early、full dose、maintenance 3. Immunosuppresant azathioprine 4.dapsone（D.D.S) 5.wilfordii preparation 6. Tetracycline plus nicotinamide 7. plasmaphoresis 8. Large dose IVIg stosstherapy