Paediatric Pyodermatitis-Pyostomatitis Vegetans Without Underlying Inflammatory Bowel Disease: A Case Report of a 3-Year-Old African Girl

Abstract

Pyodermatitis-Pyostomatitis Vegetans (PD-PSV) remains a rare disorder of unknown etiology that is often associated with underlying inflammatory bowel disease (IBD). It is also worth noting that PD-PSV is particularly rare in the paediatric population with 15.4 years as the average age of onset in reported cases. Although adults and children with IBD may exhibit similar clinical features, children often manifest with additional features of failure to thrive and delayed puberty due to the background chronic inflammatory process. In this case report, we present a case of paediatric PD-PSV without underlying inflammatory bowel disease in a 3-year-old African girl. This case presents a unique opportunity of reminding clinicians that not all cases of paediatric PD-PSV may be associated with underlying IBD at presentation.

Keywords: Crohn’s disease; azathioprine; case report; paediatric pyodermatitis-pyostomatitis vegetans; ulcerative colitis.

Figure 1

Lesions on scalp, before and after 8 weeks of azathioprine treatment.

Figure 2

Genital vegetative plagues with yellowish pustules on surface before and after 8 weeks of azathioprine treatment.

Figure 3

Before and after images of vegetative plagues with surface erosions as well as characteristic snail-track border.

Figure 4

Low power images (right) showing surface ulceration and superficial dermal infiltrate, and (left) high power image showing epidermal necrosis with predominantly dermal eosinophilic inflammatory infiltrate.