Kianindra Deisy Vania, Rosa Amelia, Pebriany Dina, Dewiyanti Wiwiek, Madjid Asnawi
Department of Dermatology and Venereology, Faculty of Medicine, Hasanuddin University, Makassar, Indonesia.
Acta Dermatovenerol Alp Pannonica Adriat. 2023 Mar;32(1):19-22.
Linear IgA bullous dermatosis (LABD) is a rare autoimmune bullous disease characterized by linear IgA deposition along the skin basal membrane. In children, LABD classically presents with a "cluster of jewels" appearance, whereas in adults the classic presentation is itchy papules with tense vesicles and bullae on an erythematous base. We report the case of a 41-year-old woman with LABD that we suspect was induced by acute myeloid leukemia presenting with multiple vesicles and bullae that coalesced, forming the typical clinical manifestation of LABD and confirmed with histopathological and direct immunofluorescence. The patient was treated with a combination of oral and topical corticosteroids with excellent results.
线状IgA大疱性皮肤病(LABD)是一种罕见的自身免疫性大疱性疾病,其特征是沿皮肤基底膜出现线状IgA沉积。在儿童中,LABD典型表现为“宝石簇”外观,而在成人中,典型表现为红斑基底上有瘙痒性丘疹及紧张性水疱和大疱。我们报告了一例41岁患有LABD的女性病例,我们怀疑其由急性髓系白血病诱发,表现为多个水疱和大疱融合,形成LABD的典型临床表现,并经组织病理学和直接免疫荧光检查确诊。该患者接受了口服和外用糖皮质激素联合治疗,效果良好。