Published on 19/03/2015 by admin
Filed under Dermatology
Last modified 19/03/2015
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Cindy E. Owen and Jeffrey P. Callen
Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports
Polyarteritis nodosa (PAN) is a necrotizing vasculitis that involves small or medium-sized arterioles. Classic PAN is characterized by fever, weight loss, cutaneous ulcers, livedo reticularis, myalgias and weakness, arthralgias or arthritis, neuropathy, abdominal pain, ischemic bowel, testicular pain, hypertension, and renal failure. Microscopic polyarteritis (MPA) involves the same-sized vessels as well as smaller vessels, and is manifest clinically as a glomerulonephritis and a pulmonary capillaritis with alveolar hemorrhage. Patients with MPA may develop small vessel vasculitis (palpable purpura), livedo reticularis with or without nodules, and/or ulcerations of the skin. Cutaneous PAN (cPAN), sometimes termed benign cutaneous polyarteritis, is characterized by livedo reticularis, nodules and ulceration, usually of the leg; it has been postulated to be a localized necrotizing arteritis that does not affect internal organs, and runs a chronic but benign course. Many reports, however, have linked cPAN to inflammatory bowel disease, streptococcal upper respiratory infection, or hepatitis B or C infection. Occasional reports have linked cPAN to antiphospholipid antibodies, cryoproteins, or antineutrophil cytoplasmic antibodies. One recent report has demonstrated that interleukin-6 is elevated in roughly 40% of patients (Kawakami, T., et al., 2012. Acta Derm Venereol 8;92 (3), 322–323). Some cases have occurred in patients treated with propylthiouracil and minocycline. cPAN appears to be more prevalent in children. Although it is generally benign, there have been reports of associated neuropathy, as well as visceral involvement.
cPAN causes pain and discomfort and may ulcerate, thereby causing disability. Therapy may include local measures, e.g., gradient pressure stockings, or systemic therapies, including systemic corticosteroids, methotrexate, azathioprine, pentoxifylline, and intravenous immuneglobulin (IVIG). Most of the reports are anecdotes or small case series.
Skin biopsy
Serology for hepatitis B and C, anti-streptolysin O antibody titers, antineutrophil cytoplasmic antibodies, antiphospholipid antibodies, and cryoproteins
Assessment for systemic involvement
Assessment for inflammatory bowel disease
Assessment for drugs that have been linked to cPAN
Daoud MS, Hutton KP, Gibson LE. Br J Dermatol 1997; 136: 706–13.
Treatment of Skin Disease Comprehensive Therapeutic Strategies 4e
