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家族的良性慢性天疱瘡(Familial Benign Chronic Pemphigus or Hailey-Hailey Disease)의 遺傳型에 對한 觀察 및 2 症例報告와 文獻考察 Genetic Observation of Familial Benign Chronic Pemphigus (Hailey-Hailey Disease) and Report of Two Cases with Review of Literatures

대한피부과학회지 1975년 13권 1호 p.61 ~ 68
金永杓, 曺質鉉,
소속 상세정보
金永杓 (  ) 
全南大學校 醫科大學 皮膚科學敎室

曺質鉉 (  ) 
全南大學校 醫科大學 皮膚科學敎室

Abstract


Familial Benign Chronic Pemphigus (Hailey-Hailey disease) is relatively rare, persistent, recurrent vesiculo-bullous and inheritable dermatitis. Forty-nine and twenty four year-old two Korean men,affecting classical form of Hailey-Hailey disease, manifested circinated or configurated form of impetiginous, vesiculo-bullous, crusted and pigmented skin ´lesions over the axillae, neck, "shoulder, lower abdomen, inguinal, inner aspect of upper portion of thigh, genito-crural and popliteal space of the skin. There were seen also actively inflammatory border that spread peripherally producing circinate and configrrate patterns, and found satellite lesions resembling cutaneous _c3ndidiasis in some areas of the lesion. However, eye lesion or lymphnode swelling was not found. Nikolsky sign ivas present in both patients. The patients had pruritus, intense or slight, over the lesion. The onset of the disease in the cases was mid-twenty and early twenty, and the durations were over twenty years and three years, respectively, and genetic observation on the families revealed that four persons in the two generations and eight persons in the four generations were affected, respectively, and ratio of male to female affected were 5 : 1. Routine laboratory examinations including bacteriological and mycological studies revealed non-specific findings. Histopathologic studies showed acantholytic and individual cells in the intra-epidermal bulla, and lacunae, villi, single basal cell lined papillae appearing at the bottom of the bulla, and so-called "dilapidated brick wall" appearance to be seen at the top of buula.
Authors discussed the gentic pattern of Familial Benign Chronic Pemphigus (Hailey-Hailey disease) in the two families and presented two cases of the disease with lraboratory examinations, histopathological findings and clinical symptoms of the patients, and confirmed that the disease is transmitted as "irregular autosomal dominant" trait, and. suessed the difference in frequency of the disease according to sex, i,e., in male were more prevalent than female, and reviewed the related literatures of the disease.

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