Familial Bening Chronic Pemphigus의 1例
A Case Familial Benign Chronic Pemphigus
박장규, 강형재, 이유신,
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박장규 ( )
서울대학교 의과대학 피부과학교실
강형재 ( )
고려병원 피부과
이유신 ( )
서울대학교 의과대학 피부과학교실
KMID : 0363219770150030333
Abstract
Sing - _he description of Familial Benign Chronic Pemphigus by Hailey-Halley, several cases of this clinical entity have reported in Korea.
Familial Benign Chronic Pemphigus is a rare hereditary acantholytic disorder, transmitted. as an irregular autosomal dominant traits, characterized by recurrent eruption of vesicles, bullae and impetiginous lesions mainly on the sides of neck, axillae.and groins symmetrically.
We presented a case of Familial Benign Chronic Femphigus in a 49 years old woman. She had suffered from these characteristic skin lesions have spread to the axillae, inframammary areas, antecubital fessae, inguinal and p;rianal areas progressively.
Moderate itching was associated with skin lesions, which were worse in summer and better in winter.
Biopsy findings showed hyperkeratosis, irregular acanthosis, multilocular suprabasal clefts of bullae with extensive acantholysis and villi formation.
Two daughters of the patient has had the same clinical features on the sides of neck since puberty.
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