摘要
目的探讨慢性家族性良性天疱疮(CFBP)的临床、病理学特点,以提高对本病的认识。方法分析经病理学确诊的16例CFBP的临床资料、组织病理及治疗。结果男10例,女6例,平均年龄37.8岁,平均病程10.56年,发病年龄3～52岁;7例有家族史。皮损好发于腹股沟、腋窝、肛周,泛发者1例;表现为在红斑/外观正常皮肤上出现水疱或丘疹伴糜烂渗液,呈扁平柔软、湿润增殖面;1例合并银屑病。组织病理学检查16例均见棘层松解如倒塌砖墙样,11例伴角化过度、松解上方灶性角化不全,13例见角化不良细胞,9例角质层/棘层上部见圆体,5例见谷粒,12例见棘层松解边缘棘层肥厚及表皮突延长;直接免疫荧光检查均阴性。口服小剂量强的松等治疗约2周可以改善临床症状,但易反复。结论本病病程长,43.75%有阳性家族史,临床易误诊为湿疹或增殖型天疱疮,组织病理学检查发现倒塌砖墙样棘层松解及直接免疫荧光检查为阴性有诊断及鉴别诊断价值,但缺乏有效治疗。
Objective To analyze the clinical features of chronic familial benign pemphigus （Hailey-Hailey disease, HHD） and have a better understanding of this disease. Methods Clinical data and histopathological char- acteristics of 16 cases with HHD were analyzed. Results Among 16 cases, 10 were male and 6 were fe- male. The mean age of patients was 37.8 years old, and the average course was 10.56 years. The age of on- set was from 3 to 52 years old. 7 patients had positive family histories. The lesions were mostly located in groins, axilla and perianal regions. However, in one patient it was widespread. Recurrent eruptions of vesi- cles and papules arose from erythematous/normal skin and later changed to erosion, exudation and some- times vegetating papillomas. One case was complicated with psoriasis. Histopathologic examinations respec- tively showed acantholysis in epidermis, with acantholytic cell giving appearance of dilapidated brick wall （16/16）, dyskeratosis cells （13/16）, round bodies （9/16）, grains （5/16）, acanthoses （12/16）. DIF of 16 cases were negative. Low dose prednisone for 2 weeks controlled the disease. Conclusion The course of HHD is long. 43.75 % of patients have positive family histories. The lesions are easily misdiagnosed as ec- zema or pemphigus vegetans. Acantholytic cells giving appearance of dilapidated brick wall, togther with negative results of DIF are valuable to make the diagnosis. But it is hard to cure.
出处
《中国皮肤性病学杂志》
CAS
北大核心
2012年第8期706-709,共4页
The Chinese Journal of Dermatovenereology
