Cicatricial pemphigoid is an extremely rare condition in children, since only 17 cases have been reported in the English literature, so far. We describe a new case in a 20-month-old boy, who is to our knowledge the youngest patient reported yet. The disorder had begun 10 months before he was referred to our department by mucosal crusted erosions of the oral and nasal cavities and conjunctivae. Cutaneous examination showed buccal erosions with limited mouth opening, entropion of the lower eyelids, trichiasis, cicatricial cornea, synechia of the nasal cavities and hypopigmented lesions of the abdomen. There were no anal or genital lesions. Cicatricial pemphigoid was confirmed by positive direct and indirect immunofluorescence on mucous biopsy. Systemic corticosteroids (2 mg/kg/day), maintained for 12 months, had led to complete healing of lesions. But due to cicatrization, synechia of the nasal cavities and corneal opacities, leading to a dramatic visual loss, have occurred. Dapsone 25 mg/day and topical ocular cyclosporine are now maintained to avoid relapse. Our review of the literature of all cases of CP showed that ocular and to a less degree, vulvar lesions are the most severe ones, due to the serious complications with scar formation.
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机译：瘢痕性天疱疮是儿童中极为罕见的疾病，因为到目前为止，英语文献中仅报道了17例。我们描述了一个20个月大男孩的新病例，据我们所知，这是迄今为止报告的最年轻的患者。这种疾病是在他因口腔，鼻腔和结膜的粘膜结s糜烂被转介到我们科室之前的10个月而开始的。皮肤检查发现颊部糜烂，张口受限，下眼睑内翻，倒睫，瘢痕角膜，鼻腔粘连和腹部色素沉着病变。没有肛门或生殖器病变。粘膜活检中直接和间接免疫荧光阳性证实了瘢痕类天疱疮。全身性糖皮质激素（2 mg / kg /天），维持12个月，已使病变完全愈合。但是由于瘢痕化，发生了鼻腔粘连和角膜混浊，导致严重的视力丧失。现在维持氨苯砜25毫克/天和局部眼用环孢素的治疗以避免复发。我们对所有CP病例的文献回顾表明，由于瘢痕形成的严重并发症，眼部和外阴部病变是最严重的。
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