Childhood Pemphigus Vulgaris during COVID-19 Outbreak Successfully Treated with Prednisone and Azathioprine: A Case Report and Literature Review

Abstract

Introduction: pemphigus vulgaris is a rare autoimmune blistering disease that involves the skin and mucous membranes and rarely occurs in pediatric age.

Methods: we present a case of childhood pemphigus in a 9-year-old patient from Burkina Faso, which initially manifested with erosive lesions symmetrically distributed in the oral cavity. After a few months, we also observed hyperchromic lesions of the back. Histopathological examination of skin samples showed intraepidermal acantholysis, while direct immunofluorescence showed deposits of complement (C3) and immunoglobulins G (IgG) in the epidermidis; an ELISA test highlighted the presence of circulating autoantibodies against desmoglein 3.

Results: the follow-up of this patient was made difficult by the advent of the COVID-19 outbreak. However, after about one year of combined therapy with systemic steroids and azathioprine the patient reached clinical remission.

Keywords: autoimmune blistering disease; childhood pemphigus; immunosuppressive therapy.

Figure 1

Clinical manifestation with erosive lesions of the oral cavity symmetrically distributed to gum (a), tongue (b) and mucous membrane of the cheeks (c). Hyperpigmented lesions on the skin arose six months later (d).

Figure 2

Histopathological examination of the mucosa at small magnification showed a detachment of the epithelium from the chorion (a). Acantholysis in histological examination of the skin, with some keratinocytes looking similar to tombstones (black arrow) (b). Positivity for C3 (c) and IgG (d) deposits showing a fishnet pattern at direct immunofluorescence on the skin biopsy.

Figure 3

Complete remission of the mucous lesions (a–c). Pigmented outcomes on the skin (d).