Pemphigus Vulgaris: A Clinical Study of 31 Cases (2004–2014) in Morocco

Abstract

Background . Pemphigus vulgaris is a rare bullous autoimmune dermatosis whose evolution and prognosis are unpredictable. Aim . The objective was to analyze long-term outcomes in patients with pemphigus vulgaris by identifying the factors that are able to influence prognosis, in particular the phenotype of pemphigus vulgaris, age at onset, multiplicity of mucosal involvement, relapse and remission rates, and survival functions. Methods . A retrospective analysis of a cohort of 31 patients followed for pemphigus vulgaris during the period from January 2004 to January 2014. Inclusion criteria were a diagnosis of pemphigus vulgaris confirmed by histopathology and direct immunofluorescence (DIF) and a period of follow-up of at least five years from the diagnosis. The following information was collected by a single investigator. Results . In total, 67.7% of patients presented a mucocutaneous pemphigus vulgaris. Male-female sex ratio was 2.4. The median duration of patient’s follow-up was estimated at 7 (6–9) years. Multiple mucosal involvement in the oral cavity and at other mucosal sites was significantly associated with severe mucocutaneous pemphigus vulgaris (<mml:math xmlns:mml="http://www.w3.org/1998/Math/MathML" id="M1"><mml:mi>p</mml:mi><mml:mo>=</mml:mo><mml:mn>0.01</mml:mn></mml:math>). Multiple relapses were significantly associated with the disease severity (<mml:math xmlns:mml="http://www.w3.org/1998/Math/MathML" id="M2"><mml:mi>p</mml:mi><mml:mo>=</mml:mo><mml:mn>0.04</mml:mn></mml:math>). Conclusion . Poor prognosis factors were severe mucocutaneous type of pemphigus vulgaris and multiple mucosal involvement in the oral cavity and at other mucosal sites.