迟发性皮肤卟啉症1例

A case of porphyria cutanea tarda

报告1例迟发性皮肤卟啉症。患者男,38岁,面部色素沉着、多毛,双手背红斑、水疱、糜烂、结痂半年余。既往有饮酒史10余年,每天约3两,发现肝功能异常2年余,余系统检查未见异常。皮肤专科查体:面部多发、弥漫性褐色色素沉着斑,双侧颧部、眶周皮肤毳毛增多,双手背见大小不等红斑、水疱、糜烂、结痂,部分好转后遗留萎缩性瘢痕。皮损组织病理学示:表皮下水疱,真皮乳头血管周围见均质红染环状沉积,少量淋巴单核细胞浸润,PAS染色示血管周围有PAS沉积,符合卟啉病皮肤改变。诊断:迟发性皮肤卟啉症。予烟酰胺片、β-胡萝卜素片口服及外用氧化锌软膏治疗后皮损好转。

A 38-year-old male presented with facial pigmentation, hairy and erythema, blisters, erosion, scab on the back of hands for more than half a year. He had drunk. Skin physical examination showed that the multiple and diffuse brown pigmented spots on the face, increased vellus hair on the bilateral zygomatic and periorbital skin, erythema, blisters, erosion and scab on the back of both hands, and atrophic scars were left after partial improvement. Histopathologic examination showed that there were subepidermal blister, homogeneous reddish-stained annular deposition around the vessels of the dermal papilla, and a small amount of lymphocyte mononuclear cell infiltration. PAS staining showed that there were PAS deposits around the vessels. He was diagnosed with porphyria catanea tarda. Symptoms were alleviated after treated with oral Nicotinamide tablets, β-carotene tablets and zinc oxide ointment.