着色性干皮病1例

A Case of Xeroderma Pigmentosum

报告着色性干皮病1例。患者,男性,7岁,因面颈部、眼结膜色素斑7年前来就诊。皮肤组织病理检查示:表皮轻度角化过度,不规则棘层增厚及萎缩,基底层色素不规则增加,真皮上部胶原纤维轻度嗜碱样变性,血管周稀疏淋巴组织细胞浸润。结合临床表现和组织病理诊断为着色性干皮病。予以避光和口服烟酰胺治疗3个月后,皮疹无增加。

We report a case with xeroderma pigmentosum. The patient was a 7 - year - old boy with pigmented macules on face, neck and conjunctiva for 7 years. Skin histopathological exami- nation revealed epidermis hyperkeratosis, unregular acanthosis and atrophy, hyperpigmentation in basal cells, basophilic degeneration of collagen fibers in upper dermis and lymphocytes infiltrate surrounding the capillaries. The diagnosis of xeroderma pigmentosum was confirmed according to clinical manifestations and histopathological findings. Skin lesions remained inactive after 3 - month treatment with photoprotection and oral nicotinamide.