Clinical manifestations and comorbidities of pemphigus: a retrospective case-control study in southern Finland

Abstract

Pemphigus is associated with several autoimmune, dermatological, and psychiatric diseases. Previous studies have reported an increasing incidence of pemphigus in Finland, particularly pemphigus foliaceus and erythematosus. The aim of this study was to determine the clinical presentation and associated comorbidities in pemphigus patients. We retrospectively assessed 66 pemphigus patients in Helsinki University Hospital and, with an age-standardised control group, performed a comparison of the studied comorbidities. The patients displayed a 0.8 female:male distribution and a mean age of 57.4 years. Pemphigus vulgaris (41%), foliaceus (30%), and erythematosus (15%) were the most common subtypes. Hypertension (30%) and dyslipidaemia (21%) were the most prevalent comorbidities. We found a statistically significant association between pemphigus and a past history of, or concurrent malignancies and atopic dermatitis (p = 0.002 and p = 0.028, respectively). No significant difference was observed in the prevalence of cardiovascular disease, asthma, chronic obstructive lung disease, type I or II diabetes mellitus, inflammatory bowel disease, depression, or anxiety. Erosions (65%), bullae (59%), and crusted lesions (55%) were observed in most patients. Half of the patients experienced pruritus before or at diagnosis. Pruritus was associated with pemphigus without mucosal involvement (p = 0.01). We found a significant association between pemphigus and atopic dermatitis and a history of malignancy. The clinical picture frequently included pruritus. These results support the findings of some recent studies of pruritus occurring more frequently in patients with pemphigus foliaceus and cutaneous pemphigus vulgaris.