# Pemphigus herpetiformis: a single-center tertiary care experience

## TL;DR

This study examines four cases of pemphigus herpetiformis, highlighting its diagnostic challenges and treatment outcomes in a single medical center.

## Contribution

The paper provides a detailed clinical and immunological analysis of a rare pemphigus variant based on a single-center experience.

## Key findings

- PH diagnosis was delayed and required high clinical suspicion due to its complex pathogenesis.
- Dapsone monotherapy was effective in only one patient, while steroids ± azathioprine achieved partial remission in three.
- Unusual immunofluorescence findings and peripheral eosinophilia were observed in some patients.

## Abstract

Pemphigus herpetiformis (PH) is a rare variant of pemphigus with a complex pathogenesis that is often difficult to diagnose. The literature generally presents data based on small case series. This study aims to broaden the understanding of PH by presenting our cases’ clinical, histopathological, and immunological characteristics.

A retrospective analysis of the medical charts of patients diagnosed with PH between 2008 and 2023 from the Dermatology Clinic at Atatürk University’s Faculty of Medicine was performed. The diagnostic criteria proposed by Kasperkiewics et al. were applied. All patients had lesional and perilesional skin biopsies for histopathologic examination and direct immunofluorescence microscopy. Circulating autoantibodies were detected by ELISA.

Four patients with a diagnosis of PH were observed. The ratio of women to men was 3:1, and the average age of the patients was 59.25 years. Pruritus was present in all patients. The oral mucosa and the scalp were affected in one and two cases, respectively. One patient showed concurrent C3 deposits at the basement membrane zone and intercellular IgG deposits on direct immunofluorescence microscopy. Peripheral eosinophilia was found in 50% of the patients. Dapsone monotherapy led to complete remission in only one patient. At least partial remission was achieved in three patients with systemic steroids ± azathioprine. No concomitant malignant disease was detected.

Establishing a diagnosis of PH may be delayed and requires a high level of clinical suspicion. Unusual direct immunofluorescence findings suggest a complex pathogenesis. A long but less severe disease course can be expected, even if dapsone monotherapy does not result in a satisfactory result in every patient.