Year: 2026
Type: article
Study design: Case Report
Abstract: Introduction: Pyoderma gangrenosum (PG) is an autoinflammatory neutrophilic dermatosis that may rarely occur in association with granulomatosis with polyangiitis (GPA). Management of refractory disease remains challenging. Case description: A 48-year-old man with a history of previous cocaine abuse was admitted due to impaired feeding in the context of a chronic hard palate perforation and a large oro-sinus fistula. During hospitalisation, he developed haematochezia and painful ulcerative skin lesions involving the right upper limb and lower extremities. Upper and lower gastrointestinal endoscopy revealed a Forrest IIc gastric lesion and a non-bleeding ileocecal ulcer. Histopathological examination of skin lesions demonstrated dense neutrophilic infiltration consistent with PG, while gastrointestinal biopsies excluded inflammatory bowel disease. An extensive evaluation excluded infection, antiphospholipid syndrome, cryoglobulinaemia, malignancy and vascular occlusive disease, all of which may mimic PG. Autoimmune screening workup yielded positive for anti-proteinase-3 antibodies (C-ANCA). The patient initially received high-dose methylprednisolone followed by mycophenolate mofetil and infliximab. Despite treatment, disease activity persisted with recurrent relapses, requiring hospitalisations. Upadacitinib was subsequently introduced, resulting in rapid clinical improvement, near-complete healing of skin lesions and successful glucocorticoid withdrawal. Conclusion: The coexistence of PG and GPA is rare and presents substantial diagnostic and therapeutic challenges. JAK inhibition with upadacitinib may represent a promising therapeutic option in refractory cases. LEARNING POINTS: Pyoderma gangrenosum may rarely coexist with granulomatosis with polyangiitis, creating significant diagnostic and therapeutic challenges.A history of cocaine abuse may complicate the diagnostic evaluation because cocaine-induced vasculitis can mimic ANCA-associated vasculitis.Upadacitinib may represent a promising steroid-sparing therapeutic option in refractory pyoderma gangrenosum associated with granulomatosis with polyangiitis.Pyoderma gangrenosum is a diagnosis of exclusion for which no confirmatory test exists; approximately 10% of patients given the diagnosis are subsequently found to have an alternative condition, so mimickers must be actively excluded before and during treatment.
DOI: 10.12890/2026_007405
Institutions: Nicosia General Hospital, University of Cyprus
Language: English
Subfield: Dermatology
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $323
OpenAlex ID: W7221818413