A case of polyarteritis nodosa limited to the right calf muscles, fascia, and skin: a case report
© Ahmed et al; licensee BioMed Central Ltd. 2011
Received: 31 May 2011
Accepted: 12 September 2011
Published: 12 September 2011
Limited polyarteritis nodosa is a rare benign disease that usually responds well to systemic corticosteroid treatment. We report a case limited to calf muscles, fascia, and skin treated with local corticosteroid therapy directed to the affected areas by ultrasound guidance.
A 36-year-old Caucasian woman presented with a 10-month history of progressive right calf pain and swelling, which were unresponsive to treatment with non-steroidal anti-inflammatory drugs and physiotherapy. An examination revealed a swollen tender right calf with indurated overlying skin. Laboratory investigations showed an erythrocyte sedimentation rate of 24 mm/hour and a C-reactive protein of 15 mg/dl. Full blood count, renal profile, and creatinine kinase level were normal. A full autoantibody screen and hepatitis B and C serology results were negative. A chest X-ray was unremarkable. Magnetic resonance imaging of the right leg revealed increased signal intensity in T2-weighted images and this was suggestive of extensive inflammatory changes of the gastrocnemius muscle and, to a lesser extent, the soleus muscle. There were marked inflammatory changes throughout the gastrocnemius muscle and the subcutaneous tissue circumferentially around the right lower leg. A biopsy of affected skin, muscle, and fascia showed histopathological features consistent with polyarteritis nodosa, including small-vessel vasculitis with fibrinoid changes in the vessel wall and intense perivascular and focal mural chronic inflammatory changes. Our patient declined treatment with oral steroids. She received a course of ultrasound-guided injections of steroid (Depo-Medrone, methylprednisolone) in the involved muscle area and commenced maintenance azathioprine with a good response.
Limited polyarteritis nodosa is rare and affects middle-aged individuals. In most cases, treatment with moderate- to high-dose corticosteroids gives symptomatic relief within one week. Resistant cases require treatment with cytotoxics or intravenous immunoglobulins. This case demonstrates response to local targeted steroid therapy as an alternative to systemic steroids.
Classic polyarteritis nodosa is a multi-system, necrotizing vasculitis of small- and medium-sized muscular arteries in which involvement of the renal and visceral arteries is characteristic . Limited forms of polyarteritis nodosa have been described, and the skin is the most common organ to be involved . Cases of polyarteritis nodosa limited to gall bladder , pancreas , female  and male  genital tracts, kidneys , and gastrointestinal tract  have also been reported. Interest in these forms is based on their prognosis, which, in general, is more benign, and their quick response to corticosteroids alone . Polyarteritis nodosa limited to calf muscles is very rare and only 14 case reports have been published. It commonly affects middle-aged individuals (average age of 40 years), and there is no significant sex variation . Laboratory markers of inflammation (erythrocyte sedimentation rate and C-reactive protein) were elevated in all previous reports. Creatinine kinase is usually within normal limits. Only two reported cases had positive autoantibodies: a positive perinuclear anti-neutrophil cytoplasmic antibody in one  and a positive anti-phospholipid antibody in the other . Unlike classic polyarteritis nodosa, which usually requires a combination of steroids and a cytotoxic drug such as cyclophosphamide for treatment , limited polyarteritis nodosa usually responds well to treatment with corticosteroids alone with symptomatic relief within one week in most cases [10, 11]. The dose of steroids used varied between 15 and 60 mg of prednisolone for initial treatment and 5 and 30 mg for maintenance. Two cases were reported to be resistant to corticosteroids but both of them responded well to intravenous immunoglobulin treatment and symptomatic response was rapid; however, one of the cases relapsed after six months and needed an increase in the oral steroid dose and the addition of methotrexate . Polyarteritis nodosa limited to calf muscles, fascia, and skin is a rare disease that runs a benign course and usually responds well to corticosteroid treatment. Resistant cases can be treated with cytotoxics such as azathioprine and methotrexate. The use of intravenous immunoglobulins is reported to induce a rapid symptomatic recovery in resistant cases, which may require cytotoxics for maintenance. The risk of progression to systemic disease is low, but close long-term follow-up of these patients may be advisable .
We describe the first case using localized corticosteroid therapy to treat polyarteritis nodosa limited to muscles, fascia, and skin, thus minimizing potential complications of systemic corticosteroid use.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
We acknowledge Ceara Walsh, who was of great assistance in revising the manuscript.
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