Cutaneous IgG4-related disease treated with dupilumab
We present a 59-year-old male with a prolonged history of severe, treatment-resistant pruritic dermatosis and associated systemic symptoms, including fatigue and diarrhea. Dermatologic examination revealed widespread erythematous-brownish papules and nodules, prompting a skin biopsy that showed dens...
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| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Sociedade Portuguesa de Dermatologia e Venereologia
2025-04-01
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| Series: | Revista da Sociedade Portuguesa de Dermatologia e Venereologia |
| Subjects: | |
| Online Access: | https://www.portuguesejournalofdermatology.com/frame_eng.php?id=217 |
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| Summary: | We present a 59-year-old male with a prolonged history of severe, treatment-resistant pruritic dermatosis and associated systemic symptoms, including fatigue and diarrhea. Dermatologic examination revealed widespread erythematous-brownish papules and nodules, prompting a skin biopsy that showed dense infiltration by immunoglobulin G4 (IgG4)-positive plasma cells, leading to a diagnosis of IgG4-related disease (IgG4-RD). The patient was treated with dupilumab, resulting in complete skin lesion resolution and significant improvement in quality of life. IgG4-RD, a rare inflammatory disease with potential multiorgan involvement, frequently challenges diagnosis due to diverse clinical presentations. This case highlights dupilumab effectiveness as a novel therapy for IgG4-RD with cutaneous involvement, offering a promising alternative for patients who do not respond well to corticosteroids.
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| ISSN: | 2182-2395 2182-2409 |