Galambrun C, Cambazard F, Clavel C, Versini P, Stéphan J L
Hôpital Nord, Saint Etienne, France.
Arch Dis Child. 1997 Sep;77(3):255-7. doi: 10.1136/adc.77.3.255.
Pemphigus foliaceus is a skin disease in which antibodies against the cell surface of keratinocytes destroy the adhesion between epidermal cells, thereby producing blisters. It is a rare disease in childhood, and treatment guidelines for juvenile pemphigus foliaceus are lacking. An 8 year old boy with pemphigus foliaceus is described. He did not respond to topical steroids, and the condition flared up when high dose oral steroids were tapered. The lesions resolved completely in four weeks on dapsone, which was maintained for nine months with no major adverse effects, except for a moderate increase of the methaemoglobin concentration at the outset of treatment. There has been no evidence of disease reactivation in more than nine months of follow up since dapsone withdrawal.
落叶型天疱疮是一种皮肤疾病，其中针对角质形成细胞表面的抗体破坏表皮细胞之间的黏附，从而产生水疱。它在儿童期是一种罕见疾病，且缺乏青少年落叶型天疱疮的治疗指南。本文描述了一名患有落叶型天疱疮的8岁男孩。他对局部用类固醇无反应，当高剂量口服类固醇逐渐减量时病情复发。使用氨苯砜治疗四周后皮损完全消退，持续使用九个月无重大不良反应，仅在治疗开始时高铁血红蛋白浓度略有升高。自停用氨苯砜后的九个多月随访中，没有疾病复发的证据。
