Pemphigus herpetiformis: a single-center tertiary care experience

Area of Science:

• Dermatology
• Immunology
• Pathology

Background:

• Pemphigus herpetiformis (PH) is a rare pemphigus variant with challenging diagnosis and complex pathogenesis.
• Existing literature often relies on small case series, limiting comprehensive understanding.

Purpose of the Study:

• To expand the understanding of PH by detailing clinical, histopathological, and immunological features.
• To analyze a cohort of PH patients to identify diagnostic and therapeutic patterns.

Main Methods:

• Retrospective analysis of medical charts for patients diagnosed with PH (2008-2023).
• Application of Kasperkiewics et al. diagnostic criteria.
• Histopathological and direct immunofluorescence (DIF) examination of skin biopsies.
• Detection of circulating autoantibodies using ELISA.

Main Results:

• Four PH patients (3 female:1 male, average age 59.25) were studied.
• All patients experienced pruritus; oral mucosa and scalp involvement occurred in one and two cases, respectively.
• One patient exhibited concurrent C3 and intercellular IgG deposits on DIF; 50% had peripheral eosinophilia.
• Dapsone monotherapy achieved complete remission in one patient; systemic steroids ± azathioprine led to partial remission in three.
• No concomitant malignant diseases were detected.

Conclusions:

• Diagnosis of PH requires high clinical suspicion and can be delayed.
• Unusual DIF findings suggest a complex underlying pathogenesis.
• PH may present a prolonged but less severe disease course, with variable responses to dapsone monotherapy.