Burden of disease during quiescent periods in patients with pemphigus

Summary Background Studies conducted using different tools have invariably observed that physical and mental components of health status are seriously compromised in patients with pemphigus. An improvement in quality of life (QoL) has been commonly observed over the treatment period. Objectives The aim of the study is to verify whether the patients' wellbeing is affected by pemphigus also in absence of cutaneous and mucosal lesions. Materials and methods The clinical records of 203 patients were analysed. A total of 47 patients were without bullae/erosions and reported a score = 0 for both the Patient Global Assessment and the Ikeda index. In order to assess the QoL we used the Skindex‐17 and the 12‐item General Health Questionnaire (GHQ‐12). Results Patients without bullae/erosions had a better QoL when compared with patients with active lesions. This difference, with a reduction of approximately 30% of the Skindex‐17 scores in the patients without lesions, was statistically significant, for both the symptoms and the psychosocial scales. The proportion of patients at risk of anxiety/depression (GHQ‐positive cases) was 44% lower in patients without lesions compared with patients with lesions. In a multiple linear regression model the presence of bullae/erosions negatively influences QoL with an average increase of Skindex‐17 symptoms and psychosocial scale scores of 11·7 and 10·6 points, respectively. Female patients had a statistically significantly worse QoL than males on the symptoms but not on the psychosocial Skindex‐17 scales. Conclusions While patients without lesions reported a better QoL than patients with bullae/erosions, their Skindex‐17 scores remained elevated. Dermatologists should be aware that a clearing of the skin manifestations does not mean ‘perfect health’ for the patient. What's already known about this topic? Pemphigus has a severe impact on quality of life. Patients with pemphigus have an increased risk of anxiety and depression. Following treatment, pemphigus may have long periods of quiescence. What does this study add? Quality of life is significantly impacted also in patients without bullae/erosions, and in women it is worse than in men. High percentages of patients are at risk of anxiety and depression even when the disease is quiescent. The burden of disease continues to be present even during periods of relative wellness.