Pemphigus erythematosus: A case series from a tertiary academic center and literature review

• Published in: Journal of cutaneous pathology Vol. 48; no. 8; pp. 1038 - 1050
• Main Authors: Hobbs, Landon K., Noland, Mary‐Margaret B., Raghavan, Shyam S., Gru, Alejandro A.
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Publishing Ltd 01.08.2021; Wiley Subscription Services, Inc
• Subjects: bullous skin disease; Demography; histopathology; immunofluorescence; Literature reviews; Patients; Pemphigus; pemphigus erythematosus; Plaques; Serology; histopathology; pemphigus erythematosus; bullous skin disease; serology; immunofluorescence
• ISSN: 0303-6987; 1600-0560
• DOI: 10.1111/cup.13992

Background Pemphigus erythematosus (PE) is a rare autoimmune skin condition with clinical, histopathological, and serological features that show overlap between lupus erythematosus and pemphigus foliaceus. It typically presents with erythematous, scaly plaques and has a female predominance. Methods After Institutional Review Board (IRB) approval, we searched the internal pathology database for “pemphigus erythematosus” in the diagnostic line between 1 January 2000 and 30 July 2020. A comprehensive chart review was performed to collect patient demographics, clinical presentation, and treatment course. We performed a review of the literature and clinical, histopathological, and serological features were collected for comparison to our case series. Results Five patients in the case series and 87 patients in the literature were diagnosed with PE. Clinical, histopathological, and serological features were consistent with what has been reported in the literature, although our cohort demonstrated a younger age at presentation, along with a higher proportion (80%) of Black patients. Of the 25 patients in the literature whose race was reported, only five patients (20%) were reported to be Black. Conclusion This is the first case series of PE that has shown an increased prevalence among middle‐aged Black patients. No specific trend in regards to race was seen in the review of the literature.