Rosenbaum M M, Esterly N B, Greenwald M J, Gerson C R
Pediatr Dermatol. 1984 Jul;2(1):13-22. doi: 10.1111/j.1525-1470.1984.tb00435.x.
Cicatricial pemphigoid has been documented previously in only four patients under the age of 20 years. We report a 6-year-old male who had erosions of the oral and genital mucosa, conjunctivitis, hoarseness, dysphagia, recurrent vomiting, and weight loss. Upper airway obstruction due to a chronically inflamed and scarred epiglottis necessitated tracheostomy. Biopsy of tissue from a solitary cutaneous lesion demonstrated a subepidermal bulla. Direct and indirect immunofluorescence confirmed a diagnosis of cicatricial pemphigoid. Therapy was begun consisting of prednisone, 2 mg per kg per day, in combination with dapsone, 2 mg per kg per day. He did not improve until the prednisone dosage was increased to 4 mg per kg per day.
瘢痕性类天疱疮以前仅在4例20岁以下的患者中有记载。我们报告1例6岁男性，有口腔和生殖器黏膜糜烂、结膜炎、声音嘶哑、吞咽困难、反复呕吐及体重减轻。由于会厌长期发炎和瘢痕形成导致上呼吸道梗阻，需行气管切开术。对单个皮肤损害的组织活检显示表皮下水疱。直接和间接免疫荧光检查确诊为瘢痕性类天疱疮。开始用泼尼松治疗，每日每千克体重2毫克，联合氨苯砜，每日每千克体重2毫克。直到泼尼松剂量增加到每日每千克体重4毫克时他才有所好转。
