Sousa Lourdes, Bajanca Rui, Cabral José, Fiadeiro Teresa
Department of Dermatology, Hospital do Desterro, and Department of Pediatric Gastroenterology, Hospital D. Estefânea, Lisbon, Portugal.
Pediatr Dermatol. 2002 Jul-Aug;19(4):336-9. doi: 10.1046/j.1525-1470.2002.00096.x.
We describe a 7-year-old boy with dermatitis herpetiformis (DH) diagnosed on clinical and histologic evidence, negative direct immunofluorescence (DIF) findings for junctional IgA deposits in uninvolved skin, positive IgA endomysial and gliadin antibodies, and jejunal biopsy revealing a gluten-sensitive enteropathy. Treatment with dapsone led to the disappearance of cutaneous lesions and pruritus within 48 hours. Demonstration of IgA immune deposits in the dermal papillae has been the only acceptable criterion for the diagnosis of dermatitis herpetiformis. However, considering several reports in the literature of DH with a negative DIF and our own case, we believe that in the absence of the characteristic DIF pattern, one needs the combination of clinical, histologic, and immunologic data to support the diagnosis of DH. We also discuss recent developments in the diagnosis of DH.
我们描述了一名7岁男孩，根据临床和组织学证据诊断为疱疹样皮炎（DH），未受累皮肤的直接免疫荧光（DIF）检查结果显示交界性IgA沉积为阴性，IgA肌内膜和麦醇溶蛋白抗体呈阳性，空肠活检显示麸质敏感性肠病。使用氨苯砜治疗后，皮肤病变和瘙痒在48小时内消失。真皮乳头中IgA免疫沉积物的显示一直是诊断疱疹样皮炎的唯一可接受标准。然而，考虑到文献中几例DIF为阴性的DH报告以及我们自己的病例，我们认为在缺乏特征性DIF模式的情况下，需要结合临床、组织学和免疫学数据来支持DH的诊断。我们还讨论了DH诊断的最新进展。
