Santos António Luís, Mota Alberto Vieira, Ramon José, Lopes José Manuel, Azevedo Filomena
Dermatology Department, Hospital de São João and Faculty of Medicine of Oporto, Portugal.
Dermatol Online J. 2007 Jul 13;13(3):17.
Bullous pemphigoid is the most common subepidermal autoimmune blistering disease. However, it is very rare in the pediatric population. A 5-month-old male infant presented with multiple bullae located on palms and soles. Complete blood count revealed relative eosinophilia . A biopsy revealed a subepidermal blister present along with a dermal mononuclear infiltrate rich in eosinophils. In focal areas collagen flame figures, were also observed. Direct immunofluorescence showed linear reactivity for C3c and IgG at the dermal-epidermal junction. IgA was not found. Complete resolution was achieved after oral deflazacort (1.5mg/kg/day) with progressive dose reduction over 2 weeks. Bullous pemphigoid is extremely rare in the infant. In our case the disease presented with an unusual localized (acral) clinical distribution and a favorable outcome.
大疱性类天疱疮是最常见的表皮下自身免疫性水疱病。然而，在儿科人群中非常罕见。一名5个月大的男婴双手掌和双足底出现多个水疱。全血细胞计数显示相对嗜酸性粒细胞增多。活检显示存在表皮下水疱以及富含嗜酸性粒细胞的真皮单核细胞浸润。在局部区域还观察到胶原纤维火焰状图像。直接免疫荧光显示在真皮-表皮交界处C3c和IgG呈线性反应。未发现IgA。口服地夫可特（1.5mg/kg/天）并在2周内逐渐减量后完全缓解。大疱性类天疱疮在婴儿中极为罕见。在我们的病例中，该疾病表现为不寻常的局限性（肢端）临床分布且预后良好。
