Cohen Daniela M, Ben-Amitai Dan, Feinmesser Meora, Zvulunov Alex
Pediatric Dermatology Unit, Schneider Children's Medical Center, Petah-Tiqva, Israel.
Pediatr Dermatol. 2009 Sep-Oct;26(5):569-74. doi: 10.1111/j.1525-1470.2009.00988.x.
Lichen planus pemphigoides is a rare autoimmune blistering disease that is characterized by evolution of vesico-bullous skin lesions in patients with active lichen planus. We describe a case of lichen planus pemphigoides in a 6-year-old boy and review the clinical and immunopathologic features of all reported cases of pediatric lichen planus pemphigoides. The mean age at onset of childhood lichen planus pemphigoides is 12 years with a male to female ratio of 3:1 and a mean lag-time between lichen planus and the development of lichen planus pemphigoides of 7.9 weeks. Vesiculo-bullous lesions were found on the extremities in all patients and there was palmoplantar involvement in about half of the cases. Direct and indirect immunofluorescence features were similar to those reported in adults. One patient had Western immunoblot data revealing antigens of 180, 230, and 200 kDa. Immunoelectron microscopy in two cases showed localization of immune deposition different from that in bullous pemphigoid. We found that topical corticosteroids or oral dapsone caused resolution of lichen planus pemphigoides without known relapse of blistering in four cases, suggesting that it might be possible to reserve oral corticosteroids as a second line of therapy in children with lichen planus pemphigoides.
扁平苔藓类天疱疮是一种罕见的自身免疫性大疱性疾病，其特征是在患有活动性扁平苔藓的患者中出现水疱性皮肤病变。我们描述了一名6岁男孩的扁平苔藓类天疱疮病例，并回顾了所有已报道的儿童扁平苔藓类天疱疮病例的临床和免疫病理特征。儿童扁平苔藓类天疱疮的平均发病年龄为12岁，男女比例为3:1，扁平苔藓与扁平苔藓类天疱疮发展之间的平均间隔时间为7.9周。所有患者的四肢均出现水疱性病变，约一半病例有掌跖受累。直接和间接免疫荧光特征与成人报道的相似。一名患者的免疫印迹数据显示有180、230和200 kDa的抗原。两例患者的免疫电子显微镜检查显示免疫沉积的定位与大疱性类天疱疮不同。我们发现，局部使用皮质类固醇或口服氨苯砜可使4例扁平苔藓类天疱疮消退，且水疱无复发，这表明对于患有扁平苔藓类天疱疮的儿童，有可能将口服皮质类固醇留作二线治疗药物。
