Granulomatous vasculitis refers to inflammation of the cutaneous blood vessels in which the inflammatory infiltrate consists primarily of histiocytes and giant cells. This constellation of findings has been associated with lymphoproliferative disorders, systemic vasculitis, autoimmune disease, and infection. Granulomatous vasculitis is most commonly associated with lymphoma and systemic vasculitides, such as granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, giant cell arteritis, and Takayasu arteritis. Less commonly, lymphomatoid granulomatosis (LYG), leukemia, sarcoidosis, Crohn disease, ulcerative colitis, hepatitis C infection, tuberculosis, postherpetic eruptions, peripheral neuropathy, and rheumatoid arthritis also have been associated with granulomatous vasculitis.

The symptoms associated with granulomatous vasculitis depend on the size of the blood vessels affected. Large vessel involvement may produce symptoms of vascular insufficiency such as claudication of the jaw and limbs, transient visual disturbances, syncope, and weak pulses. Small and medium vessel involvement typically presents with skin findings, such as palpable purpura, tender subcutaneous nodules, and ulcerations. Small vessel involvement may also produce upper airway symptoms such as nasal crusting and polyposis, in addition to other symptoms depending on the site of involvement. Systemic symptoms are often present in patients with granulomatous vasculitis including fever, fatigue, weight loss, and arthralgias, in addition to other symptoms depending on the underlying disease.