Publicação
Cutaneous IgG4-related disease treated with dupilumab
| Resumo: | Abstract 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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| Autores principais: | Pestana,Mafalda |
| Outros Autores: | João,Alexandre; Palma-Carlos,Susana; Leiria-Pinto,Paula; João,Ana L. |
| Assunto: | Immunoglobulin G4-related disease Cutaneous Dupilumab |
| Ano: | 2025 |
| País: | Portugal |
| Tipo de documento: | relatório |
| Tipo de acesso: | acesso aberto |
| Instituição associada: | Fundação para a Ciência e Tecnologia |
| Idioma: | inglês |
| Origem: | SciELO Portugal |
| Resumo: | Abstract 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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