Brivet F, Drüeke T, Guillemette J, Zingraff J, Crosnier J
Nephron. 1978;20(5):258-66. doi: 10.1159/000181232.
Among 500 patients on maintenance hemodialysis, 6 patients (5 young women and a 49-year-old man) developed bullous dermatosis, 2-54 months after initiating dialysis treatment. The skin lesions occurred mainly in sunlight-exposed areas, and 4 out of the 6 patients showed increased cutaneous fragility in response to trauma. Skin biopsy revealed subepidermal blisters for all of them, and skin immunofluorescence studies were negative for 2 patients. No increase in fecal or red cell coproporhyrin and protoporphyrin levels was found in any of the 6 patients. The syndrome was clinically and histologically indistinguishable from porphyria cutanea tarda.
在500例维持性血液透析患者中，6例患者（5名年轻女性和1名49岁男性）在开始透析治疗2至54个月后出现大疱性皮肤病。皮肤病变主要发生在阳光暴露部位，6例患者中有4例在受到创伤后皮肤脆性增加。皮肤活检显示所有患者均有表皮下水疱，6例患者中有2例皮肤免疫荧光检查呈阴性。6例患者中无一例粪便或红细胞粪卟啉和原卟啉水平升高。该综合征在临床和组织学上与迟发性皮肤卟啉症无法区分。
