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Granulomatosis with polyangiitis (GPA) is a small-to-medium vessel vasculitis that can affect the skin, respiratory tract, kidneys and other organs. A rare cutaneous manifestation of GPA is pyoderma gangrenosum (PG)-like ulcerations, which can have debilitating and disfiguring consequences. We report the case of a man in his 40s with refractory PG-like ulcerations secondary to GPA, not responsive to conventional immunosuppression, who was successfully treated with rituximab.

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Patients with granulomatosis with polyangiitis occasionally present with cutaneous manifestations, which are important clues for the early diagnosis. Although pyoderma gangrenosum-like ulcers are rarely observed, a unique case with unusual clinical features is presented herein. A 75-year-old woman with positive proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA) repeatedly developed aseptic abscesses on the abdomen, buttock, lower legs, and forearms.

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Granulomatosis with polyangiitis mimicking multisystem pyoderma gangrenosum: A case report.

SAGE Open Med Case Rep

December 2024

Division of Clinical Dermatology and Cutaneous Science, Department of Medicine, Dalhousie University, Halifax, NS, Canada.

Granulomatosis with polyangiitis, a type of small-medium vessel vasculitis, can pose diagnostic challenges due to its diverse clinical manifestations. We present a case that mimicked multisystem pyoderma gangrenosum with cutaneous ulceration, renal sparing, and antineutrophil cytoplasmic antibody negativity. Although antineutrophil cytoplasmic antibody is positive in approximately 90% of cases of granulomatosis with polyangiitis, its negativity should not exclude the diagnosis.

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Article Synopsis
  • MENin-KMT2A inhibitors (MENi) are new drugs being tested for treating specific types of acute myeloid leukemia (AML), particularly KMT2Ar and NPM1 cases.
  • A 71-year-old woman treated with the MENi revumenib (REV) for relapsed NPM1 AML developed a serious skin condition resembling pyoderma gangrenosum after two weeks.
  • This case is significant as it is the first documented instance of MENi causing this type of skin condition, emphasizing the need to monitor for severe skin-related side effects in patients undergoing treatment with MENi.
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Article Synopsis
  • Pyoderma gangrenosum is a rare skin disease that causes painful ulcers with specific characteristics and can occur alongside other conditions like rheumatoid arthritis and inflammatory bowel disease.
  • A unique case was reported involving a 67-year-old obese female who had both pyoderma gangrenosum and IgA vasculitis, leading to large, rapidly expanding skin wounds.
  • Treatment with a TNF-α inhibitor (adalimumab) successfully reduced the ulceration and promoted healing after initial treatment with oral prednisolone.
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