Dermatologic manifestations of granulomatosis with polyangiitis: A case report and literature review

Abstract

Granulomatosis with polyangiitis (GPA) is an ANCA-associated, systemic vasculitis of small and medium-sized blood vessels. GPA causes inflammation and destruction to the vessel wall and eventual tissue and organ damage. It classically affects the tissues and vasculature of the sinuses, lungs, and kidneys. The organ damage results in epistaxis, cough, hemoptysis, shortness of breath and/or even kidney failure. Cutaneous manifestations are rare, but have been reported. We discuss the case of a 27-year-old African American female who presented with skin lesions on her bilateral forearms and hands, several years after diagnosis and treatment for GPA. The lesions were isolated papules, located on bilateral elbows, palms, and the lateral edge of the 3rd digit on her left hand. The lesions were intensely pruritic and non-painful. A skin biopsy of the lesions showed neutrophilic and granulomatous inflammation with nuclear debris, a largely non-specific histopathologic finding. An extensive autoimmune work up revealed elevated c-ANCA/PR3-ANCA levels supporting a diagnosis of cutaneous GPA. GPA is diagnosed by a combination of clinical signs and symptoms, serologic testing, and histology from biopsy of affected organs. Patient’s who meet the criteria should be tested for anti-neutrophil cytoplasmic antibody (ANCA), specifically c-ANCA/PR3-ANCA. A positive ANCA is supportive for GPA diagnosis. However, a negative ANCA does not rule out disease. Patients with cutaneous findings suggestive of GPA and positive c-ANCA/PR3-ANCA serologic testing should be closely followed up, which will lead to overall better prognosis, improved health outcomes and reduced patient and health care expenses.