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Case Reports

Abstract

Pyodermatitis-pyostomatitis vegetans (PPV) is a rare mucocutaneous dermatosis characterized by oral lesions and vegetating plaques of flexural areas, most commonly associated with inflammatory bowel disease (IBD), particularly ulcerative colitis.¹ Herein, we present the case of a 79-year-old female with IBD who presented with oral and skin lesions, which was found to be associated with elevated antineutrophil cytoplasmic antibodies (ANCAs), and ultimately diagnosed as PPV.  A 79-year-old female with a history of IBD presented with ulceration of the tongue with hemorrhage and erythematous skin lesions with granulation under the breast and groin for the past few months. Biopsy of the skin lesion showed focal epidermal necrosis, neutrophilic/eosinophilic spongiosis with intraepidermal bullae and acantholysis, and superficial/mid-dermal inflammation with scattered neutrophils and numerous eosinophils. Direct immunofluorescence (DIF) showed non-specific staining, although enzyme-linked immunosorbent assay was negative for pemphigus. Microbiology was negative for herpes simplex virus type 1 and 2 and varicella zoster virus. The patient was found to have elevated C-antineutrophil cytoplasmic antibodies (ANCA) (1:80), P-ANCA (1:640), and anti-proteinase-3 (PR3), and given the clinical picture was diagnosed with PPV. Topical corticosteroid treatments improved skin lesions but not oral lesions.  Positive ANCAs have been associated with autoimmune disorders, such as IBD and ANCA vasculitis. Oral findings in these conditions include PPV and strawberry gingivitis, respectively, and may have similar clinical presentations. Physicians should be aware that the differential diagnoses for ANCA-positive oral lesions should not exclude PPV in association with IBD and vegetative cutaneous lesions until otherwise ruled out. References: 1. https://pubmed.ncbi.nlm.nih.gov/32621702/