Genetic Observation of Familial Benign Chronic Pemphigus ( Hailey - Hailey Disease ) and Report of Two Cases with Review of Literatures.
- Author:
Young Pio KIM
;
Jill Heyeun CHO
- Publication Type:Original Article
- MeSH:
Axilla;
Candidiasis, Cutaneous;
Dermatitis;
Family Characteristics;
Female;
Humans;
Male;
Neck;
Pemphigus;
Pemphigus, Benign Familial*;
Pruritus;
Shoulder;
Skin;
Thigh
- From:Korean Journal of Dermatology
1975;13(1):61-68
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Familial Benign Chronlc 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 abdornen, 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 configurate patterns, and found satellite lesions resembling cutaneous candidiasis in some areas of the lesion. However, eye lesion or lymphnode swelling was not found. Nikolsky sign was 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 blula. 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 laboratory examinations, histopathological findings and clinical symptoms of the patients, and confirmed that the disease is transmitted as "irregular autosomal dominant" trait, and stressed the difference in frequency, of the diseae according to sex, I.e., in male were more prevalent than female, and reviewed the related literatures af the disease.
