Kim Ashley S, Sundnes Olav
Department of Dermatology, Oslo University Hospital, Oslo, Norway.
Institute of Clinical Medicine, University of Oslo, Oslo, Norway.
Case Rep Dermatol. 2025 May 3;17(1):185-190. doi: 10.1159/000546155. eCollection 2025 Jan-Dec.
Linear IgA dermatosis (LAD) is a rare subepidermal autoimmune blistering skin disorder characterized by the linear deposition of IgA along the basal membrane. It affects primarily young children and adults, and is the most common bullous disease in the paediatric population. Dapsone represents the mainstay of treatment, to which the majority of patients show excellent initial responses with long-term remission. In recalcitrant cases, sulfonamides (sulfapyridine, sulfasalazine, sulfamethoxypyridazine) are considered second-line options either as monotherapy or in conjunction with dapsone. Most published cases of sulfonamides in childhood LAD report use of sulfapyridine or sulfamethoxypyridazine, with no published reports on sulfasalazine use in young children.
We present a case of a 1-year-old child with confirmed LAD who did not respond adequately to dapsone alone. Sulfapyridine is not available in Norway, while sulfasalazine is accessible and considered a safe option for other autoimmune disorders. The addition of sulfasalazine resulted in rapid complete remission.
This case thus supports sulfasalazine as a pragmatic, accessible alternative to sulfapyridine as the second-line treatment in childhood LAD.
线状IgA大疱性皮病（LAD）是一种罕见的表皮下自身免疫性大疱性皮肤病，其特征是IgA沿基底膜呈线状沉积。它主要影响幼儿和成人，是儿科人群中最常见的大疱性疾病。氨苯砜是主要的治疗药物，大多数患者对其初始反应良好且能长期缓解。对于难治性病例，磺胺类药物（磺胺吡啶、柳氮磺胺吡啶、磺胺甲氧嗪）被视为二线选择，可单独使用或与氨苯砜联合使用。大多数已发表的儿童LAD中使用磺胺类药物的病例报告使用的是磺胺吡啶或磺胺甲氧嗪，尚无关于幼儿使用柳氮磺胺吡啶的报道。
我们报告一例确诊为LAD的1岁儿童，该儿童单独使用氨苯砜治疗效果不佳。挪威没有磺胺吡啶，而柳氮磺胺吡啶可以获得，并且被认为是治疗其他自身免疫性疾病的安全选择。加用柳氮磺胺吡啶后迅速完全缓解。
因此，该病例支持柳氮磺胺吡啶作为儿童LAD二线治疗中磺胺吡啶的实用、可及替代药物。
