Three Cases of Familial Benign Chronic Pemphigus / 대한피부과학회지

ABSTRACT

Familial Renign Chronic Femphigus(FRCP) is rare, persistent and vesicobullous genodermatoai. Three casee of FRCP with typical clinical and histapathological findings are reported. The first case of FBCP was 27-year-oId man who had recurrent episodes of grou vesicles and rust with miId pruritue at the posterior neck, in both axillae and in inguinal area for 10 years. The second case was 49-year-old woman with skin lesions of recurrent persistent. vesicles, pustules on the erythematous skin, crust and lichenification on the flexural area of extrimeties, axillae, ingumal and neck area for 10 yeara. The third case was 49-year-old male patient who had recurrent pruritic grouped vesicles with crust on the neck and axillary area. In all three patients, skin eruptions usually began and or aggravated in the spring or summer and tend to heal spontaneously in the autumn and winter and showed positive Nikolsky sign. The only one case of the reported three casea had familiaI history of skin lesions similar to FRCP. Histopat,holag;ic finding of biopsy specimens from the vesicular eruption showed auprabasal vesiculation with acantholysis, lacunae and villi formation in all 3 cases.