Department of Dermatology, Hospital Clinic de Barcelona, CAPSBE, Barcelona, Spain.
Department of Pediatrics, CAPSBE, Barcelona, Spain.
J Pediatr Adolesc Gynecol. 2022 Apr;35(2):171-173. doi: 10.1016/j.jpag.2021.10.010. Epub 2021 Nov 2.
Autoimmune bullous diseases in childhood are a diagnostic challenge.
We present the case of an 11-year-old girl with recurrent vulvar erosions since early childhood. She had been referred to a child abuse unit under the suspicion of sexual abuse. She responded well to dapsone and topical corticosteroids.
Our review focuses on previously reported cases of pemphigoid (bullous or mucous membrane) in childhood with exclusively genital involvement. We also summarize mucous membrane pemphigoid cases diagnosed during childhood. There seems to be a differentiated form of pemphigoid predominantly affecting girls with exclusively vulvar involvement and with good prognosis. Dermatologic evaluation and a skin biopsy with direct immunofluorescence are key to diagnosing a mucous membrane pemphigoid. Further antigenic studies are needed to nosologically classify the disease properly.
儿童自身免疫性大疱性疾病是一种诊断挑战。
我们报告了一例 11 岁女孩，自幼儿期开始反复出现外阴糜烂。曾因疑似性虐待被转介至儿童虐待专科。她对氨苯砜和局部皮质类固醇反应良好。
我们的综述重点关注了以前报道的儿童大疱性类天疱疮（大疱性或黏膜性）病例，这些病例仅涉及生殖器。我们还总结了儿童期诊断的黏膜性类天疱疮病例。似乎有一种不同形式的大疱性类天疱疮，主要影响女孩，仅累及外阴，预后良好。皮肤科评估和皮肤活检直接免疫荧光检查是诊断黏膜性类天疱疮的关键。需要进一步的抗原研究来正确对疾病进行分类。
