POLYARTERITIS NODOSA

By Gary M. White, MD

Polyarteritis nodosa Several punched-out ulcers.


Polyarteritis nodosa (PAN) is a rare necrotizing, systemic vasculitis that targets medium-sized arteries. It is typically ANCA-negative and is not associated with glomerulonephritis (renal involvement may occur, but results from medium vessel involvement compromising vascular supply).

Clinical

Palpable purpura, punched out ulcers, tender subcutaneous nodules and livedo reticularis may occur in PAN. Less commonly, peripheral gangrene may develop.

Some patients may have a chronic, relapsing course, no visceral disease and a good, long term prognosis--which some have called cutaneous PAN [JAMA Derm 2015;551].

Workup

A biopsy deep enough to get representative vessels is necessary. A related and possibly causative infection should be sought e.g. Hepatitis B, HIV and in children, recent Streptococcal infection. Other diseases which have been associated with PAN include SLE, inflammatory bowel disease, familial Mediterranean fever and hairy cell leukemia. A thorough HP should be done as well as laboratory investigations including CBC, ESR, electrolytes, creatinine, LFT's, urine analysis, throat culture, ASO, Hepatitis B and HIV serology. Tuberculosis should be excluded as Tb infection can mimic PAN.

Treatment

Referral to a rheumatologist is in order. In the past, prednisone has been used with or without cyclophosphamide, and NSAID's.

Additional Pictures

A purpuric lesion and a punched-out ulcer.
Polyarteritis nodosa

References

Indian Dermatology Online Journal

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