Pyostomatitis Vegetans With Orofacial and Vulvar Granulomatosis in a Pediatric Patient

Area of Science:

• Dermatology
• Gastroenterology
• Pediatrics

Background:

• Pyostomatitis vegetans (PSV) is a rare oral manifestation often linked to inflammatory bowel disease (IBD).
• While commonly associated with ulcerative colitis, PSV can also occur in Crohn disease (CD).
• This case highlights the diagnostic challenges of PSV in pediatric patients.

Observation:

• A 7-year-old girl presented with PSV and multiple extraintestinal findings suggestive of CD.
• These included cheilitis granulomatosa, perianal and vulvar edema with noncaseating granulomas, and anal skin tags.
• Elevated fecal calprotectin was noted during a cutaneous flare, but gastrointestinal involvement remained undetected for four years.

Findings:

• The case underscores the potential for PSV and associated extraintestinal manifestations to precede overt gastrointestinal findings of IBD in children.
• Biopsies confirmed noncaseating granulomas, supporting a diagnosis of Crohn disease despite the absence of endoscopic evidence.
• This presentation emphasizes the importance of a comprehensive evaluation in pediatric cases of PSV.

Implications:

• Early recognition of PSV and associated extraintestinal signs may prompt earlier IBD investigation in pediatric patients.
• Management strategies for pediatric PSV without confirmed IBD require careful consideration and literature review.
• This case contributes to the limited data on managing pediatric PSV, particularly when gastrointestinal involvement is delayed or absent.