Cutaneous polyarteritis nodosa

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Cutaneous polyarteritis nodosa

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

image

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.

Specific investigations

Cutaneous periarteritis nodosa: a clinicopathological study of 79 cases.

Daoud MS, Hutton KP, Gibson LE. Br J Dermatol 1997; 136: 706–13.

This retrospective analysis of 79 patients evaluated the clinical and histological features of cPAN and attempted to identify any clinical, pathological, and immunological differences that may distinguish those cases likely to have a prolonged course. During the course of their illness 39 patients had ulcers. Women were affected more than men. Painful nodules on the lower extremities, with edema and swelling, were the most common clinical finding; 22% of patients had some evidence of neuropathy. Most of the laboratory findings were non-specific. There was no evidence for hepatitis B infection in the 37 patients tested, and hepatitis C infection was present in only one of the 20 patients tested. Five patients had inflammatory bowel disease (four had Crohn disease and one had ulcerative colitis). Ten patients had rheumatoid arthritis. Most patients (60%) had no associated medical condition. The disease course was prolonged but benign, and systemic PAN did not develop in any patient. The ulcerative form of disease was more prolonged and frequently associated with neuropathy. Therapy was varied, and the patients with non-ulcerative disease responded better than those with ulcers. Observations suggested that agents such as corticosteroids, azathioprine, pentoxifylline, and hydroxychloroquine were effective in individual patients.

This study highlights the associations that might occur in up to 40% of patients, the fact that neuropathy is relatively common, and that the course and response to treatment are dependent on the presence of ulceration.

First-line therapies

imageLocal measures, i.e., support stockings, local wound care E
imageNon-steroidal anti-inflammatory drugs, penicillin (if antecedent streptococcal infection), systemic corticosteroids E
imageImmunosuppressants: azathioprine, methotrexate, mycophenolate mofetil, mizoribine E

Second-line therapies

imageIntravenous immunoglobulin E
imagePentoxifylline E

Third-line therapies

imageTamoxifen E
imageInfliximab E
imageTonsillectomy D
imageWarfarin E

[/level-membership-for-dermatology-category][not-level-membership-for-dermatology-category]

Cutaneous polyarteritis nodosa

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

image

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.