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 Blster intraepidermal Acantholysis in bullae
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. choice drug，early、full dose、maintenance 3. Immunosuppresant
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