Pemphigus and pemphigoidPemphigus and pemphigoid

(pemphigus)(pemphigus)

definitiondefinition ：： autoimmune skin and mautoimmune skin and mucous membrane vesiculobullous diucous membrane vesiculobullous disease is characterized with sease is characterized with

acantholysis,bullae formation in the iacantholysis,bullae formation in the intraepidermal areas caused by ntraepidermal areas caused by

intercellular antibodies deposited in sintercellular antibodies deposited in stratum spinosumtratum spinosum

classificationclassification ：： four types tfour types two specieswo species

vulgaris type vulgaris type pemphigus vulgarispemphigus vulgaris （（ bullae at underlayer bullae at underlayer

of acanthocytesof acanthocytes ）） pemphigus vegetanspemphigus vegetans

foliaceus type foliaceus type pemphigus foliaceuspemphigus foliaceus （（ bullae at superficialbullae at superficial layer of acanthocyteslayer of acanthocytes ）） pemphigus erythematosuspemphigus erythematosus

pathogenesypathogenesyEvidence of autoimmune diseaseEvidence of autoimmune disease ：： Ig deposited between acanthocytes of skin lesiIg deposited between acanthocytes of skin lesi

onon Pemphigus antibodies are present in serumPemphigus antibodies are present in serum antibody titer related with pathogenetic coantibody titer related with pathogenetic co

nditionndition Plasmapheresis removing pemphigus antibodiPlasmapheresis removing pemphigus antibodi

es is useful in a short termes is useful in a short term pemphegus antibodies transfer fetus across plpemphegus antibodies transfer fetus across pl

acentaacenta High titer pemphigus antibodies repeatedly intHigh titer pemphigus antibodies repeatedly int

radermal injection induced acantholysisradermal injection induced acantholysis

Clinical featuresClinical featuresIncidence of pemphigus per year0.5~3.2/100,000Incidence of pemphigus per year0.5~3.2/100,0001. 1. Pemphigus vulgarisPemphigus vulgaris Usually occurs in middle-ageUsually occurs in middle-age （（ age of 60age of 60 ）） Blister and erosion usually appear on oral mucoBlister and erosion usually appear on oral muco

sasa thin-walled,flaccid bullae or enlarged erosion sthin-walled,flaccid bullae or enlarged erosion s

ufaceuface Nikolsky sign positive Nikolsky sign positive Lesions appear in the scalp,face,chest and backLesions appear in the scalp,face,chest and back

2. 2. pemphigus vegetanspemphigus vegetans

HallopeauHallopeau

NenmannNenmann

The lesions limited in axillaeThe lesions limited in axillae 、、 grgroin oin 、、 under breastunder breast 、、 perineuperineum, etc.m, etc.

3. 3. pemphigus foliaceuspemphigus foliaceus Lesions are at upper layer of acantLesions are at upper layer of acant

hocyteshocytes

Thin-walled bullae or porous thick Thin-walled bullae or porous thick crustscrusts

Lesions aggravate after exposed tLesions aggravate after exposed to sunlighto sunlight

pemphigus erythematosuspemphigus erythematosus

lesions appear most commonly in thlesions appear most commonly in the scalpe scalp 、、 faceface 、、 superior part of trsuperior part of trunkunk

Erythema and flaccid bullaErythema and flaccid bulla

Nikolsky sign Nikolsky sign （＋）（＋）

Immunologic testImmunologic test

direct immunofluorecencedirect immunofluorecence （（ DIFDIF ） ） ：：

in acantholytic areasin acantholytic areas

IgGIgG 、 、 IgMIgM 、、 IgAIgAor or CC33 depostion depostion

Indirect immunofluorecenceIndirect immunofluorecence（（ IIFIIF ）） Circulating intercellular antibodies can be Circulating intercellular antibodies can be

demonstrated in 80demonstrated in 80 ～～ 9090 ％ ％ of patients’ sof patients’ serum with pemphigus vulgariserum with pemphigus vulgaris

Mainly IgGMainly IgG

Antibody titer often parallel disease activity Antibody titer often parallel disease activity

histopathologyhistopathology

pemphigus vulgarispemphigus vulgaris Blster intraepidermalBlster intraepidermal

Acantholysis in bullaeAcantholysis in bullae （（ pemphigus cellpemphigus cell ））

Diagnosis and antidiastoleDiagnosis and antidiastolePemphigus vulgarisPemphigus vulgaris （（ 11 ）） Usually occurs in the elderlyUsually occurs in the elderly （（ around age around age

of 60of 60 ）） Blister and erosion usually appear on oral Blister and erosion usually appear on oral

mucosamucosa thin-walled,flaccid bullae or enlarged erosthin-walled,flaccid bullae or enlarged eros

ion sufaceion suface Nikolsky sign positiveNikolsky sign positive Lesions appear in the scalp,face,chest and Lesions appear in the scalp,face,chest and

backback

Diagnosis and antidiastoleDiagnosis and antidiastole

Pemphigus vulgarisPemphigus vulgaris （（ 22 ）） DIF shows Ig deposited intercellularDIF shows Ig deposited intercellular IIF shows pemphigus antibodiesIIF shows pemphigus antibodies Pathology shows blister intraepiderPathology shows blister intraepider

malmal ，， acantholysisacantholysis

treatmenttreatment

1. General treatment1. General treatment Surpportive therapySurpportive therapy 、、 vitamin anvitamin an

d protein supplementd protein supplement Transfusion and eleltrolyte balancTransfusion and eleltrolyte balanc

ee Attending raw surfacesAttending raw surfaces ，， prevent iprevent i

nfectionnfection Caution the complicationCaution the complication

2. corticosteroid2. corticosteroid choice drugchoice drug ，， earlyearly 、、 full dosfull dos

ee 、、 maintenancemaintenance3. Immunosuppresant3. Immunosuppresant azathioprine or cytoxanazathioprine or cytoxan4.dapsone4.dapsone （（ D.D.SD.D.S))

5.wilfordii preparation5.wilfordii preparation

6. 6. plasmaphoresisplasmaphoresis7. Large dose IVIg stosstherapy7. Large dose IVIg stosstherapy 0.4g / kg / d ×30.4g / kg / d ×3 ～～ 5d5d

pemphigoidpemphigoid

definitiondefinition An autoimmunity dermatosis mainly oAn autoimmunity dermatosis mainly o

ccurs in aged people ccurs in aged people ，， characterized characterized with blister formation intraepidermalwith blister formation intraepidermal

classificationclassification

Bullous pemphigoidBullous pemphigoid （（ BPBP ）） Localized pemphigoidLocalized pemphigoid 小疱型类天疱疮小疱型类天疱疮 结节痒疹型天疱疮结节痒疹型天疱疮 Pleomorphous pemphigoidPleomorphous pemphigoid Cicatricial pemphigoidCicatricial pemphigoid

Pemphegoid clinical featuresPemphegoid clinical features（（ BPBP ）） usually occurs in the elderlyusually occurs in the elderly （（ over agover ag

e of 70e of 70 ）） thic-walled tense bullae or blisterthic-walled tense bullae or blister Maily apear in axillaeMaily apear in axillae 、、 groingroin Histopathology shows bullae subepideHistopathology shows bullae subepide

rmalrmal DIF shows CDIF shows C33 and/or and/or IgGIgG linear deposit linear deposit

ed in the basement of the membrane zed in the basement of the membrane zoneone

treatmenttreatment

1. General treatment1. General treatment High protein ingestHigh protein ingest Prevent skin secondary Prevent skin secondary

infectioninfection Eliminate malignant Eliminate malignant

tumor tumor

2. 2. corticosteroidcorticosteroid choice drugchoice drug ，， earlyearly 、、 full dosefull dose 、、

maintenancemaintenance3. Immunosuppresant3. Immunosuppresant azathioprine azathioprine 4.dapsone4.dapsone （（ D.D.SD.D.S))5.wilfordii preparation5.wilfordii preparation6. Tetracycline plus nicotinamide6. Tetracycline plus nicotinamide7. 7. plasmaphoresisplasmaphoresis8. Large dose IVIg stosstherapy8. Large dose IVIg stosstherapy