AI Article Synopsis
- Linear IgA bullous dermatosis (LABD) is an autoimmune skin disorder characterized by blistering and the presence of IgA deposits at the basement membrane.
- Diagnosis involves skin pathology tests and direct immunofluorescence to confirm the diagnosis, with symptoms including tense blisters and intense itching.
- Treatment often starts with dapsone, but in a reported case resistant to other therapies, dupilumab was used to manage itching while awaiting HLA-B*1301 gene test results to prevent serious drug reactions.
Article Abstract
Linear IgA bullous dermatosis (LABD) is an acquired autoimmune subepidermal blistering disorder. Diagnosis always relies on skin pathology and direct immunofluorescence (DIF), with typical linear deposits of IgA along the basement membrane zone (BMZ). The typical clinical manifestation is tense bullae arranged like the "string of pearls" companied with severe pruritus. Dapsone is often considered first-line therapy for LABD, and it is necessary to test the HLA-B*1301 gene to prevent the occurrence of dapsone-induced hypersensitivity syndrome (DHS). Here we report a case of LABD resistant to corticosteroid and sulfasalazine, while waiting for HLA-B*1301 gene test results, dupilumab was used to control severe pruritus.
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Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11330783 | PMC |
| http://dx.doi.org/10.3389/fimmu.2024.1409556 | DOI Listing |
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