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Granulomatous vasculitis - Skin
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Granulomatous vasculitis - Skin

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Contributors: Lily Changchien MD, Nikki Levin MD, Jeffrey D. Bernhard MD, Noah Craft MD, PhD, Lindy P. Fox MD, Lowell A. Goldsmith MD, MPH, Michael D. Tharp MD
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Synopsis

Granulomatous vasculitis refers to inflammation of the cutaneous blood vessels, in which the inflammatory infiltrate consists primarily of histiocytes and giant cells. This is a non-specific finding that 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, leukemia, sarcoidosis, Crohn's disease, ulcerative colitis, hepatitis C infection, tuberculosis, post-herpetic 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.

Codes

ICD10CM:
L95.8 – Other vasculitis limited to the skin

SNOMEDCT:
9177003 – Granulomatous vasculitis of skin

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Henoch-Schönlein purpura – Direct immunofluorescence will show IgA deposition in vessel walls. Also, patients may present with arthralgias and abdominal pain.
  • Infection-mediated leukocytoclastic vasculitis – Biopsy will show necrotizing vasculitis.
  • Medication-induced leukocytoclastic vasculitis (such as anti-TNF agents, G-CSF, NSAIDs, and penillicins) – Biopsy will show a necrotizing vasculitis.
  • Leukocytoclastic vasculitis associated with inflammatory disorders – Biopsy will show necrotizing small vessel vasculitis, and patient may have a positive ANA, rheumatoid factor, low C3 or C4.
  • Polyarteritis nodosa – Biopsy will show a segmental necrotizing vasculitis of small vessels and medium arteries. Celiac / mesenteric / renal angiography may show microaneurysms.
  • Pyoderma gangrenosum – Biopsy will show neutrophilic infiltrate.
  • Thrombosis (eg, antiphospholipid syndrome, sepsis, disseminated intravascular coagulation, or thrombotic thrombocytopenic purpura) – Skin biopsy will show intravascular thrombi.
  • Cryoglobulinemic vasculitis – Biopsy will show granular deposits of IgM and C3 in the papillary dermis. Serum cryoglobulins will likely be positive.
  • Cholesterol emboli – Biopsy will show elongated clefts within the lumens of small vessels, often with thrombi.
  • Calciphylaxis – Biopsy will show perivascular deposition of calcium.

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 Updated: 10/26/2017
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Granulomatous vasculitis - Skin
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Granulomatous vasculitis : Claudication, Palpable purpura, Symmetric extremities
Clinical image of Granulomatous vasculitis
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