College of Medicine, University of Nebraska Medical Center, Omaha, Nebraska, USA.
Department of Dermatology, College of Medicine, University of Nebraska Medical Center, Omaha, Nebraska, USA.
Pediatr Dermatol. 2024 Sep-Oct;41(5):897-899. doi: 10.1111/pde.15622. Epub 2024 Apr 15.
Mucous membrane pemphigoid is a rare autoimmune disease affecting mucosal surfaces. Pediatric cases are exceptionally rare, one subtype being vulvar pemphigoid. Juvenile vulvar pemphigoid can be challenging to diagnose due to its rarity and subtle initial symptoms. We present a case of an 8-year-old girl successfully diagnosed early in the disease course via histopathology, and immunofluorescence. Detecting MMP can be complex due to variations in epitope binding typically not included in commercial ELISA assays, necessitating comprehensive workup. Missed diagnosis may lead to progression to systemic involvement with severe consequences; thus, timely diagnosis and treatment are crucial.
黏膜类天疱疮是一种罕见的自身免疫性疾病,影响黏膜表面。儿科病例极为罕见,其中一种亚型为外阴部类天疱疮。由于其罕见性和初期症状不明显,儿童外阴部类天疱疮的诊断具有挑战性。我们报告了一例 8 岁女孩的病例,通过组织病理学和免疫荧光检查在疾病早期成功诊断。由于表位结合的变化通常不包括在商业 ELISA 检测中,因此检测 MMP 可能很复杂,需要进行全面的检查。漏诊可能导致疾病进展为全身性疾病,后果严重;因此,及时诊断和治疗至关重要。