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伴有“珍珠串征”的婴儿大疱性类天疱疮

Infantile bullous pemphigoid with "string of pearls sign".

作者信息

Raposo Inês, Machado Susana, Sampaio Rita, Selores Manuela

机构信息

Department of Dermatology, Centro Hospitalar do Porto, Portugal.

出版信息

Dermatol Online J. 2017 Jul 15;23(7):13030/qt3fw4d2t2.

Abstract

Bullous pemphigoid (BP) is an immune mediated bullous disease that is manifested by urticarial plaques with superimposed subepidermal blisters and significant pruritus. It is generally found in the elderly, but is rare in the pediatric population. A 5-month-old girl previously diagnosed with hand-foot-mouth disease was examined in our dermatology department owing to vesicles and bullae, initially located to the hands and feet, which progressed with new lesions. Tense vesicles and bullae distributed in an annular string of pearls pattern on the abdomen and facial and cervical regions were noted. Histologic and immunologic findings were consistent with the diagnosis of infantile BP. Disease control was obtained with oral prednisolone and dapsone; the patient was still in clinical remission 6 months after treatment cessation. The differential diagnosis of the clinical presentation of the lesions in our patient is of note, given that this blistering pattern is frequently reported in association with linear IgA bullous dermatosis.

摘要

大疱性类天疱疮(BP)是一种免疫介导的大疱性疾病,表现为荨麻疹斑块伴表皮下大疱及显著瘙痒。该病通常见于老年人,在儿童群体中罕见。一名先前诊断为手足口病的5个月大女孩因手部和足部最初出现水疱和大疱,并伴有新的皮损进展,前来我院皮肤科就诊。腹部、面部和颈部可见紧张性水疱和大疱呈环形串珠状分布。组织学和免疫学检查结果符合婴儿型BP的诊断。口服泼尼松龙和氨苯砜后病情得到控制;停药6个月后患者仍处于临床缓解期。鉴于这种水疱模式常与线状IgA大疱性皮病相关,故对我们患者皮损临床表现的鉴别诊断值得关注。

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