URTICARIA PIGMENTOSA: IMPACTS ON QUALITY OF LIFE AND THE ROLE OF PHARMACOTHERAPY IN SYMPTOM MANAGEMENT

Abstract

Urticaria pigmentosa is the most prevalent form of cutaneous mastocytosis in adults, characterized by the abnormal accumulation of mast cells in the dermis, manifesting as hyperpigmented lesions, intense pruritus, and, in some cases, systemic symptoms resulting from the release of inflammatory mediators. Although life expectancy is generally preserved, the condition imposes a considerable physical, emotional, and social burden, with direct repercussions on quality of life. This study constitutes a narrative literature review based on publications indexed in PubMed/MEDLINE, PMC, and Orphanet from 2021 to 2026, aiming to analyze the disease's impact on quality of life and to evaluate the effectiveness of the main pharmacotherapeutic strategies available. Results indicate that domains such as sleep, emotional well-being, social functioning, and work capacity are most affected. Regarding treatment, H1 and H2 antihistamines remain first-line options; omalizumab stands out in refractory cases; and tyrosine kinase inhibitors — midostaurin and avapritinib — represent significant advances, particularly in the management of indolent systemic mastocytosis. It is concluded that integrating effective pharmacological interventions into clinical care substantially improves patient well-being, underscoring the need for an individualized and multiprofessional therapeutic approach.

References

AKIN, C.; AROCK, M.; CARTER, M. C.; GEORGE, T. I.; VALENT, P. Mastocytosis. Nature Reviews Disease Primers, v. 11, n. 1, p. 30, 2025. Disponível em: https://pubmed.ncbi.nlm.nih.gov/40274818/ Acesso em: 08 maio 2026 DOI: https://doi.org/10.1038/s41572-025-00611-8 DOI: https://doi.org/10.1038/s41572-025-00611-8

CHANG, J. et al. Omalizumab as an emerging treatment for severe refractory cutaneous mastocytosis: a systematic review of the adult and paediatric literature. Journal of the American Academy of Dermatology, v. 91, n. 3 (Suppl), p. AB116, 2024. Disponível em: https://www.jaad.org/article/S0190-9622(24)02105-4/fulltext Acesso em: 08 maio 2026 DOI: https://doi.org/10.1016/j.jaad.2024.07.1064

COSTA, Rafael Capelo; MOLINA, Juliana D'Andrea; MORGADO DE ABREU, Marilda Aparecida Milanez. Urticária pigmentosa no adulto: tratamento bem-sucedido em associação com fototerapia. Revista de Medicina, São Paulo, Brasil, v. 99, n. 4, p. 400–404, 2020. Disponível em: https://revistas.usp.br/revistadc/article/view/143076 Acesso em: 08 maio 2026 DOI: https://doi.org/10.11606/issn.1679-9836.v99i4p400-404 DOI: https://doi.org/10.11606/issn.1679-9836.v99i4p400-404

CRIADO, P. R.; CRIADO, R. F. J.; MIOT, H. A.; ABDALLA, B. M. Z.; MARCHIORO, H. Z.; BONAMIGO, R. R. Chronic spontaneous urticaria: update on pathogenesis and therapeutic implications. Anais Brasileiros de Dermatologia, v. 100, p. 501198, 2025. DOI: Disponível em: https://doi.org/10.1016/j.abd.2025.501198 Acesso em: 08 maio 2026 DOI: https://doi.org/10.1016/j.abd.2025.501198 DOI: https://doi.org/10.1016/j.abd.2025.501198

FAZIO, M.; BOTTARO, A.; NASSO, M. E.; STAGNO, F.; ALLEGRA, A. Skeletal involvement in systemic mastocytosis: pathophysiology, clinical management, standards of care, and novel therapeutic strategies. Cells, v. 15, n. 3, p. 307, 2026. Disponível em:https://pmc.ncbi.nlm.nih.gov/articles/PMC12896522/ Acesso em: 08 maio 2026 DOI: https://doi.org/10.3390/cells15030307 DOI: https://doi.org/10.3390/cells15030307

GOTLIB, J. et al. Practical management of adverse events in patients with advanced systemic mastocytosis receiving midostaurin. Expert Opinion on Biological Therapy, v. 21, n. 4, p. 487-498, 2021. Disponível em: https://pubmed.ncbi.nlm.nih.gov/33063554/ Acesso em: 08 maio 2026 DOI: https://doi.org/10.1080/14712598.2021.1837109 DOI: https://doi.org/10.1080/14712598.2021.1837109

SPOLAK-BOBRYK, N.; NIEDOSZYTKO, M.; JASSEM, E.; CHEŁMIŃSKA, M.; LANGE, M.; MAJKOWICZ, M.; NEDOSZYTKO, B.; BORCHET, J. Assessing quality of life in patients with mastocytosis: development of the disease-specific questionnaire. Postępy Dermatologii i Alergologii, v. 38, n. 6, p. 1044-1051, 2021. Disponível em: https://pmc.ncbi.nlm.nih.gov/articles/PMC8802953/ Acesso em: 8 maio 2026 DOI: https://doi.org/10.5114/ada.2021.108435 DOI: https://doi.org/10.5114/ada.2021.108435

MACRI, A.; SALEH, H. M. Urticaria pigmentosa. In: STATPEARLS PUBLISHING. StatPearls. Treasure Island: StatPearls Publishing, 2026. Disponível em: https://www.ncbi.nlm.nih.gov/books/NBK482503/ Acesso em: 08 maio 2026.

MESA, R. A. et al. Patient-reported outcomes among patients with systemic mastocytosis in routine clinical practice: results of the TouchStone SM Patient Survey. Cancer, v. 128, n. 20, p. 3691-3699, 2022. Disponível em: https://pubmed.ncbi.nlm.nih.gov/35996873/ Acesso em: 08 maio 2026 DOI: https://doi.org/10.1002/cncr.34420 DOI: https://doi.org/10.1002/cncr.34420

NÖLDEKE, P. et al. Avapritinib reduces symptoms and mast cell burden in systemic mastocytosis. Allergy, Asthma & Clinical Immunology, v. 21, n. 1, p. 40, 2025. Disponível em: https://pmc.ncbi.nlm.nih.gov/articles/PMC12442258/ Acesso em: 08 maio 2026 DOI: https://doi.org/10.1186/s13223-025-00986-z DOI: https://doi.org/10.1186/s13223-025-00986-z

PYATILOVA, P.; SIEBENHAAR, F. Measuring symptom severity and quality of life in mastocytosis. Immunology and Allergy Clinics of North America, v. 43, n. 4, p. 751-762, 2023. Disponível em: https://pubmed.ncbi.nlm.nih.gov/37758411/ Acesso em: 08 maio 2026 DOI: https://doi.org/10.1016/j.iac.2023.04.003 DOI: https://doi.org/10.1016/j.iac.2023.04.003

SCHMIDT, T. J. et al. Health-related quality of life and health literacy in patients with systemic mastocytosis and mast cell activation syndrome. Orphanet Journal of Rare Diseases, v. 17, n. 1, p. 295, 2022. Disponível em: https://pmc.ncbi.nlm.nih.gov/articles/PMC9336039/ Acesso em: 08 maio 2026 DOI: https://doi.org/10.1186/s13023-022-02439-x DOI: https://doi.org/10.1186/s13023-022-02439-x

USTUN, C. et al. Systemic mastocytosis: current status and challenges in 2024. Blood Advances, v. 9, n. 9, p. 2048-2062, 2025. Disponível em: https://pubmed.ncbi.nlm.nih.gov/39853317/ Acesso em: 08 maio 2026 DOI: https://doi.org/10.1182/bloodadvances.2024012612

How to Cite

Cristina Ferreira da Silva, E., Oliveira, J. da S. ., Ribeiro, K. R. S., Santos, K. N. dos, & Costa, L. F. da. (2026). URTICARIA PIGMENTOSA: IMPACTS ON QUALITY OF LIFE AND THE ROLE OF PHARMACOTHERAPY IN SYMPTOM MANAGEMENT. RECIMA21 - Revista Científica Multidisciplinar - ISSN 2675-6218, 7(6), e768094. https://doi.org/10.47820/recima21.v7i6.8094