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Granulomatous vasculitis
Other Resources UpToDate PubMed

Granulomatous vasculitis

Contributors: Haya Raef MS, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

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.

Codes

ICD10CM:
L95.9 – Vasculitis limited to the skin, unspecified

SNOMEDCT:
9177003 – Histiocytic vasculitis of skin
I77.6 – Arteritis, unspecified

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Differential Diagnosis & Pitfalls

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed:03/02/2021
Last Updated:03/22/2021
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Granulomatous vasculitis
Granulomatous vasculitis : Claudication, Palpable purpura, Skin ulcers
Clinical image of Granulomatous vasculitis
Reddish-brown plaques, some with overlying superficial desquamative scale, on the lower legs.
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