An atypical case of granulomatosis with polyangiitis in a 15-year-old girl presenting with pyoderma gangrenosum–like ulcers on the face

Granulomatosis with polyangiitis (GPA) is an uncommon form of antineutrophil cytoplasmic antibodies-associated necrotizing vasculitis characterized by symptoms affecting the ears, nose, and throat, along with respiratory issues and kidney involvement. Although GPA is extremely rare among children, delayed diagnosis can have fatal consequences. We report a unique instance of GPA in a 15-year-old girl exhibiting solitary pyoderma gangrenosum-like ulcers on the face. After an extensive evaluation, the patient was diagnosed with GPA and demonstrated an excellent response to dual therapy combining rituximab and corticosteroids. Although exceptionally uncommon, clinicians must consider GPA as a possible condition in the differential diagnosis of individuals presenting with pyoderma gangrenosum-like ulcers.