Pénfigo vulgar: estudio de cohorte retrospectivo de sus características clínicas,tratamientos empleados y evolución

BackgroundPemphigus vulgaris (PV) is an uncommon, serious disease that is treated with systemic corticosteroids and corticosteroid-sparing agents.ObjectivesTo describe and analyze the demographic and clinical characteristics of patients with PV.Material and methodsRetrospective cohort study of adults diagnosed with PV over a period of 12 years.ResultsPV presented with mucosal lesions in 20 of the 32 patients studied (63%); the most common site was the oral mucosa followed by the vulva. Mucosal involvement was more common in women (P = .03). Lesions were found at more than 1 mucosal site in patients whose disease began in the mucosa, independently of age or sex (P = .003). Disease onset before the age of 40 years was associated with generalized skin lesions (P = .003), a need for corticosteroid-sparing therapy (P = .05), and refractory PV (P = .02). Azathioprine was the most widely prescribed corticosteroid-sparing agent (in 22 patients). Eight patients (25%) were dependent on corticosteroids and disease recurred in 26 (81%). Complete remission, with or without treatment, was achieved in 15 patients (47%). Patients remained disease-free for a median of 14 months, and 2 patients died (6%).ConclusionOnset before the age of 40 years could be a sign of poor prognosis in patients with PV, as it was significantly associated with a higher risk of generalized skin involvement, a need for corticosteroid-sparing therapy, and refractory disease.