OBJECTIVES: Pemphigus and pemphigoid (P/P) are rare, autoimmune blistering diseases; real-world data is needed to better understand diagnosis, treatment patterns, and quality of life in these difficult to diagnose patients. The P/P Registry was launched in March 2017 to fulfill this unmet need and bring awareness to healthcare providers.

METHODS: As of September 2018, the registry has enrolled 259 patients. Patient demographics, diagnosis type, treatment patterns, and quality of life (QoL) measurements including physical, mental health, and activity participation were analyzed for patients who provided diagnosis and clinical information.

RESULTS: Patient respondents were predominately female (75%, 104/138), mean age at diagnosis was 49.1 years (SD 14.3 years), 79% (109/138) were white, 80% (110/138) were US-based, and 61% (76/138) had commercial insurance. 52% (61/118) of patients were diagnosed with pemphigus vulgaris, 19% (22/118) with mucous membrane pemphigoid, 14% (16/118) with bullous pemphigoid, and the remaining 15% (19/118) with other P/P subtypes. Of patients reporting medication information, 61% (54/89) took oral corticosteroids, 55% (49/89) topical corticosteroids, 43% (38/89) immunosuppressants, and 25% (22/89) rituximab. Quality of life was reported as fair or poor for 22% (17/76) of patients. Patients reported frequent fatigue (41%, 32/77) and depression (17%, 13/79). 25% (19/76) of patients indicated pain significantly interfered with their enjoyment of life and 34% (23/67) reported poor or very poor quality of sleep.

CONCLUSIONS: This patient registry study showed significant patient burden and negative impact of P/P on patients’ physical and mental health. Future studies of this growing longitudinally collected registry data are warranted to help raise P/P disease awareness amongst medical, research, and patient community.