1、大皰性皮膚病 BULLOUS DERMATOSIS概 述Introduction大皰性皮膚病是指一組發(fā)生在皮膚黏膜以大皰為基本損害的皮膚病 。病因包括自身免疫和遺傳等，前者包括天皰瘡、大皰性類天皰瘡，后者包括家族性慢性良性天皰瘡和大皰性表皮松解癥。天 皰 瘡PEMPHIGUS天皰瘡是一組累及皮膚和粘膜的自身免疫性表皮內(nèi)大皰病。共同的特征有：薄壁、松弛易破的大皰；組織病理為表皮內(nèi)水皰；免疫病理顯示角質(zhì)細胞間IgG、IgA、IgM 或C3的網(wǎng)狀沉積。Pemphigus. ruptured bullae with crustPemphigus. HistopathologyPemphigus. Ac

2、antholysisimmunofluorescence(IF) IgG IgA IgM & C3 netty deposition at interkeratinocyte病因及發(fā)病機理患者血中有抗角質(zhì)形成細胞間物質(zhì)抗體，其與角質(zhì)形成細胞間物質(zhì)結(jié)合，使細胞釋放纖維蛋白酶原激活物，導致細胞間粘合物質(zhì)降解，引起表皮棘層細胞松解。 Circulating intercellular antibodies(pemphigus antibody 天皰瘡抗體) are present in the patients serum, and antibody titers often parallel di

3、sease activity.角質(zhì)形成細胞間物質(zhì)？ 橋粒desmosomes臨床分型Classification 尋常性天皰瘡pemphigus vulgaris 增殖性天皰瘡pemphigus vegetans 落葉性天皰瘡pemphigus foliaceous 紅斑性天皰瘡pemphigus erythematous尋常性天皰瘡 pemphigus vulgaris 口腔黏膜常受累 好發(fā)于頭頸、胸背部 松弛性大皰、壁薄、易破 尼氏征陽性 The Nikolsky sign is positive pemphigus vulgaris occurs with equal frequency

4、 in men and women ,usually their fifth and sixth decades. It is rare in young persons. The mouth lesions appear first in 60% of cases. generalized bullous phase in most patients occurs some 5 or more months after the onset of oral lesions.增殖性天皰瘡pemphigus vegetans 好發(fā)于腋窩、乳房下、腹股溝、外陰、肛周等部位創(chuàng)面上有肉芽增殖尼氏征陽性有

5、臭味 pemphigus vegetans is characterized by flaccid bullae that bacome erosions and form fungoid(蕈狀的) vegetations(增殖) or papillomatous(乳頭瘤樣) proliferations(增生), especially in body foids(皺褶),and reducing the foul(惡臭) odor(氣味). 落葉性天皰瘡pemphigus foliaceous好發(fā)于中老年人；口腔黏膜受累少見；皰壁極薄、易破，形成黃褐色油膩性葉片性痂和鱗屑；尼氏征陽性。 pe

6、mphigus foliaceus is a relatively mild ,chronic variety of pemphigus characterized by flaccid bullae and localized or generalized exfoliation(剝脫). PF begins with small, relatively flaccid bullae, the bullae soon rupture to form erosion which covered with filemot(黃褐色)greasy(油膩性) crusts.紅斑性天皰瘡pemphigu

7、s erythematous好發(fā)于頭面部、胸背上部紅斑、水皰尼氏征陽性黏膜損害少見 The leision are erythematous and thickly crusted , bullous or even hyperkeratotic(角化過度). These are usually localized on the nose, cheeks, and earsthe sites frequently affected by lupus erythematosis mucous membranes lesion are rarely seen.診斷依據(jù) Diagnosistypic

8、al clinical features 松弛性水皰、易破、不易愈合； 尼氏征陽性 可伴有口腔黏膜受損組織病理為表皮內(nèi)水皰；免疫病理顯示角質(zhì)形成細胞間IgG、IgA、IgM或C3的沉積。Biopsy material(活檢) Because the bullae of pemphigus become large and flaccid in a short time .it is important that a small, early, intact blister be secured.治 療Treatment一般治療General treatment ：營養(yǎng)支持療法糖皮質(zhì)激素： 早期

9、使用、足量控制、合理減量、長期維持。免疫抑制劑：常與激素聯(lián)合用藥 血漿置換療法抗感染療法局部用藥Treatment Systemic therapy Corticosteroid therapy is the standard treatment of choice. the sooner the diagnosis is established and the sooner treatment is given, the more favorable the prognosis. the therapeutic effects are estimated by the number of n

10、ew vesicles per day and the rate of healing of the new lesion, Nikolsky sign, pemphigus antibody titers. medication is continued until clinical disease is suppressed and pemphigus antibody disappears from the serum.Treatment Systemic therapy Corticosteroid therapy prednisone area dosage 10% 3040mg/d

11、 3050% 6080mg/d 50% 80100mg/d or more原則：早期使用、足量控制、合理減量、長期維持notice The clinician should remember that today the risk of death in pemphigus from the side effects of oral prednisone is greater than the risk of death from the disease itself.大皰性類天皰瘡 bullous pemphigoid是一種自身免疫性表皮下大皰病。表現(xiàn)為水皰壁厚、不易破，組織病理為表皮下水皰

12、，免疫病理示基底膜帶IgG和C3線狀沉積發(fā)病機理患者血清中有抗基底膜帶的自身抗體；靶抗原為半橋粒上的BP230和BP180. IgG & C3 linear deposition on basement membrane zone(BMZ). Circulating BMZ antibodies of IgG class are present in the patients serum. No close correlation exists between the titer of antibodies and clinical disease activity.臨床表現(xiàn)clinical

13、features好發(fā)于50歲以上的中老年人好發(fā)部位：軀干、四肢伸側(cè)、腋窩、腹股溝緊張性水皰Thick-walled, large, tense, subeppidermal bullae尼氏征陰性The Nikolsky sign is negativeBullous pemphigoid. ImmunopathologyIgG & C3 linear deposition on basement membrane zone診 斷Diagnosis緊張性水皰、壁厚不易破組織病理為表皮下水皰免疫病理示基底膜帶IgG和C3線狀沉積治 療Treatment 糖皮質(zhì)激素 Corticosteroid therapy is the standard treatment of choice. prednisone 0.51.0mg/(kg.d) 免疫抑制劑 氨苯砜Dapsone