Buffoni S, Salvai M
Divisione di Pediatria, Ospedale E. Agnelli, Pinerolo, Torino.
Minerva Pediatr. 1995 Oct;47(10):409-15.
We report a case of chronic bullous dermatosis of children, a benign dermatologic disease of infancy of unknown etiology, characterized, on IIF, by linear deposits of immunoglobulins in the Basement Membrane Zone of cutis, and clinically by a ring shaped disposition of bullae (rosette-like). This work concerns a ten year old girl admitted to the pediatric ward at E. Agnelli Hospital of Pinerolo (Turin) about one year ago, who started having perioral bullous eruption, subsequently spread all over the body skin, with general symptoms of fever and itching. Round erythematous plaques, crusts, and a ring disposition of sausage-shaped subepidermal bullae were suggestive of CBDC. Diagnosis was confirmed by cutaneous biopsy and DIF. Corticosteroids, given for about six mths., and DDS in the last four mths. of treatment, quickly changed the course of the disease, which has not recurred for about one year. Differential diagnosis with other bullous dermatoses of 2nd and 3rd childhood has been addressed to bacterial and viral diseases as well as to toxico-allergic and autoimmune diseases, the latter rarely seen by Pediatricians. Among those we discuss on clinical and anatomopathological aspects of Dermatitis herpetiformis, Erythema multiforme, Pemphigus and Pemphigoid, with regard to different anatomic sites of bullous lesions and to pathogenesis involved immunoglobulins and skin antigens.
我们报告一例儿童慢性大疱性皮肤病,这是一种病因不明的婴儿期良性皮肤病,在间接免疫荧光检查中,其特征为免疫球蛋白在皮肤基底膜带呈线性沉积,临床特征为水疱呈环形分布(玫瑰花结样)。本研究涉及一名10岁女孩,约一年前入住皮内罗洛(都灵)E. 阿涅利医院儿科病房,最初出现口周大疱性皮疹,随后蔓延至全身皮肤,并伴有发热和瘙痒等全身症状。圆形红斑、结痂以及腊肠形表皮下水疱的环形分布提示为儿童慢性大疱性疾病。皮肤活检和直接免疫荧光检查确诊了该疾病。给予约6个月的皮质类固醇治疗,并在治疗的最后4个月使用氨苯砜,迅速改变了疾病进程,至今约一年未复发。与儿童第二和第三阶段的其他大疱性皮肤病的鉴别诊断涉及细菌和病毒疾病以及毒过敏性和自身免疫性疾病,后者儿科医生很少见到。在这些疾病中,我们从临床和解剖病理学方面讨论了疱疹样皮炎、多形红斑、天疱疮和类天疱疮,涉及大疱性病变的不同解剖部位以及发病机制中涉及的免疫球蛋白和皮肤抗原。