Abstract

Granulomatosis with polyangiitis (GPA) is a rare, systemic disease that can affect multiple organ systems including the skin. While most cutaneous lesions appear concurrently or after systemic symptoms, skin findings can be the initial manifestation of the disease. It is thus important to recognize the cutaneous manifestations of GPA, which can be a valuable clue to the diagnosis. We report a 30-year-old male who presented with ulcerated nodules on his palms and elbows and worsening hemoptysis. CT scan of the chest revealed scattered, rounded, air space opacities with central ground glass and peripheral consolidated rim. While bronchoscopy was being scheduled, a dermatology visit revealed scattered round, firm, focally ulcerated papules and nodules ranging in size from 3mm to 1cm on the palms and elbows. Shave biopsy of a nodule revealed a palisaded granulomatous infiltrate surrounding degenerated connective tissue containing neutrophils. No vasculitis was present. Special stains were negative for microorganisms. These findings were consistent with GPA. Subsequent laboratory evaluation revealed positive anti-neutrophil cytoplasmic antibodies with a c-ANCA titer of 1:320, and a proteinase-3 antibody (p-ANCA) of over 8 IU/mL. The patient was admitted to intensive care after bronchoscopy for worsening of his hemoptysis. 

This patient’s rocky course highlights the role the dermatopathologist can play in identifying a rare but deadly systemic disease presenting in the skin.