局限于外阴和肛周的慢性家族性良性天疱疮1例

A Case of Familial Benign Chronic Pemphigus Localized on Vulva and Perianal Region

患者女,25岁。外阴及肛周散在分布水疱伴瘙痒和疼痛1年。皮损组织病理检查示:角化过度,表皮增生,基底细胞层上水疱,棘层松解细胞见于表皮全层或表皮的下半部,大部分棘层松解细胞只是轻度分离,犹如坍塌的砖墙,可见角化不良的棘层松解细胞。真皮乳头水肿,浅层血管周围以淋巴细胞为主的浸润。诊断:慢性家族性良性天疱疮。

A 25-year-old female presenled with blisters on the vulva and perianal region with itching and pain for one year. Skin lesions histopathology showed that there were hyperkeratosis, epidermal hyperplasia for several blisters on the basal cell layer,acantholysis resembling a ＂dilapidated brick wall＂. Dyskeratotic and acantho- lytie cell was observed. There was edema in the dermal papilla and shallow perivascular infiltration of lymphoeytes. Diagnosis：familial benign chronic pemphigus.