Instituto Nacional de Salud del Niño de Breña.
Instituto Nacional de Salud del Niño San Borja. Lima, Perú.
Bol Med Hosp Infant Mex. 2023;80(Supl 1):77-81. doi: 10.24875/BMHIM.22000118.
Acquired epidermolysis bullosa is a rare and chronic autoimmune subepidermal bullous disease characterized by the formation of autoantibodies against type VII collagen. Presentation in childhood is rare and with several manifestations.
We report the case of a 12-year-old female patient who presented bullous and polymorphic lesions on the chest and extremities of several months of evolution. Due to the characteristics of the skin lesions, a histopathological and direct immunofluorescence study was conducted, confirming the diagnosis of acquired epidermolysis bullosa. Subsequently, corticosteroid and dapsone treatment was administered, with favorable clinical response during follow-up.
Acquired epidermolysis bullosa is unusual in pediatric age, so it should be considered in the differential diagnosis of other congenital and acquired bullous diseases of childhood. The definitive diagnosis is performed through an immunofluorescence, study, which allows for rapid and effective treatment to control the disease and avoid permanent sequelae.
获得性大疱性表皮松解症是一种罕见的慢性自身免疫性表皮下大疱性疾病，其特征是针对 VII 型胶原形成自身抗体。儿童期发病罕见，且有多种表现。
我们报告了一例 12 岁女性患者，其胸部和四肢出现水疱和多形性皮损，病程有数月。鉴于皮肤损伤的特征，进行了组织病理学和直接免疫荧光研究，确诊为获得性大疱性表皮松解症。随后，给予皮质类固醇和氨苯砜治疗，随访期间临床反应良好。
获得性大疱性表皮松解症在儿科年龄不常见，因此应在儿童先天性和获得性大疱性疾病的鉴别诊断中考虑到该病。通过免疫荧光研究可明确诊断，从而能够快速、有效地进行治疗，控制疾病并避免永久性后遗症。
