Harper P, Thunell S, Hultcrantz R, Ros A M, Wennersten G
Porfyricentrum Sverige.
Lakartidningen. 1998 Jul 8;95(28-29):3195-9.
Porphyria cutanea tarda (PCT) is probably the most common of the porphyrias. The development of skin fragility and blisters are the symptoms that generally bring the patient to the notice of the dermatologist. During the past decade the disease has been recognised as being of heterogeneous aetiology, and a pathogenetic classification has been proposed. The significance of subtyping for the choice of management strategy is currently appreciated, as is the need of close monitoring owing to the risk of the common PCT-associated liver conditions. Preferably the PCT patient should be managed by a dermatologist and a hepatologist working in concert and supported by a specialised porphyria laboratory. The use of a structured management protocol should be considered.
迟发性皮肤卟啉症(PCT)可能是最常见的卟啉病。皮肤脆弱和水疱的出现是通常会引起皮肤科医生注意的症状。在过去十年中,该病被认为病因具有异质性,并提出了一种发病机制分类。目前人们认识到亚型分类对管理策略选择的重要性,以及由于常见的PCT相关肝脏疾病风险而进行密切监测的必要性。PCT患者最好由皮肤科医生和肝病专家协同管理,并得到专门的卟啉病实验室的支持。应考虑使用结构化管理方案。