RESUMO
BACKGROUND: Pemphigus is a rare but severe autoimmune disease caused by autoantibodies directed against desmosomes, and clinically characterized by bullae and painful erosions of the skin and mucous membranes. The two major subtypes, vulgaris and foliaceus, are distinguished by the depth of the cleavage plane in the epidermis. Very few studies have investigated the quality of life (QoL) of patients with pemphigus, all reporting a strong impact on physical and emotional status. OBJECTIVES: We sought to achieve an exhaustive description of health status in a large sample of patients with pemphigus, assess the impact on QoL, and define a minimum set of QoL tools for clinical practice. METHODS: In all, 139 patients with pemphigus enrolled at our bullous skin diseases department from February 2007 to February 2008 were given the Medical Outcome Study 36-item short form health survey questionnaire to assess the health status, the Skindex-29 to evaluate the impact of dermatologic-specific aspects, and the 12-item General Health (GH) Questionnaire to detect patients with psychological problems. Clinical severity of the disease was assessed by a dermatologist by the Physician Global Assessment index and the Ikeda index. RESULTS: A strong impact of pemphigus on health status was observed, especially in women and older patients, and in patients with mucocutaneous involvement. A significant association between disease severity and lower Medical Outcome Study 36-item short-form questionnaire values was also observed. Patients with pemphigus showed a markedly impaired overall QoL compared with healthy control subjects on all 3 Skindex-29 scales (symptoms mean scores 37 vs 8, in patients and control subjects, respectively; emotions 37 vs 14; functioning 33 vs 4; P < .001); disease severity was also significantly associated with Skindex-29 scores, on all 3 scale scores for both Physician Global Assessment and Ikeda values (P < .05). GH Questionnaire positivity, reflecting probable minor psychiatric nonpsychotic conditions, such as depression and anxiety, was detected in 39.7% of patients. LIMITATIONS: The small sample size in the different treatment groups prevented a more detailed analysis, failing to highlight an association between treatment type and QoL impairment. CONCLUSIONS: In this study we described a strong impact of pemphigus on patients' QoL both for the dermatology-specific and the GH aspects. The prevalence of patients with GH Questionnaire positivity was also very high (almost 40%). The introduction of the proposed minimal set of QoL evaluation tools would provide additional useful information to guide clinicians in the treatment of these patients.