- 2025, Volume 83 - Number 2
- Published: March 27, 2025
Cutaneous IgG4-related disease treated with dupilumab
Mafalda Pestana 1, Alexandre João 2, Susana Palma-Carlos 3, Paula Leiria-Pinto 3, Ana L. João 4
1 Department of Dermatology and Venereology, Centro Hospitalar Universitário de Lisboa Central, Lisbon, Portugal; 2 Department of Dermatology and Venereology, Unidade Local de Saúde de São José, Alameda Santo António dos Capuchos, Lisbon, Portugal; 3 Centro Clínico Académico de Lisboa; Department of Immunoallergology, Unidade de Saúde Local de São José. Lisbon, Portugal; 4 Department of Dermatology and Venereology, Hospital de Santo António dos Capuchos, Unidade Local de Saúde São José; Centro Clínico Académico de Lisboa. Lisboa, Portugal
Mafalda Pestana, Alexandre João, Susana Palma-Carlos, Paula Leiria-Pinto, Ana L. João
La información completa de afiliaciones y autor de correspondencia está disponible en la versión original en PDF.
*Correspondence: Alexandre João, Email not available
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.
