Cutaneous Polyarteritis Nodosa without any subcutaneous nodules but with Pyoderma Gangrenosum like ulcers: A Rare Case Report
Abstract Cutaneous Polyarteritis Nodosa (cPAN) is a rare form of vasculitis of medium and small sized vessel of unknown etiology, first described by Kussmaul and Maier in 1866. The average age of onset is approximately 50 years. In both adults and children, males appear to be more commonly affected than females. cPAN is characterized by disease affecting primarily the skin without any major organ involvement. cPAN is demonstrated purpura, ulcerations, and tender recurrent subcutaneous nodules on the lower extremities as well as postinflammatory hyperpigmentation. Systemic symptoms may include fever, rash, joint pain and myopathy. Diagnosis is confirmed by histopathologic evidence of necrotizing inflammation of the medium and small-sized arteries. Treatment for cPAN includes the use of topical or intralesional corticosteroids for limited disease, systemic corticosteroids for extensive disease, and nonsteroidal anti-inflammatory drugs for symptomatic relief. cPAN is difficult to control and often requires longer courses of prednisone and steroid-sparing agents such as colchicine. Untreated polyarteritis nodosa has a poor prognosis, with a 5-year survival of 13%. Treatment improves this substantially, with current 5-year survival rates of approximately 80%. Subcutaneous nodules precede skin ulceration in a case of cPAN in 80% of times. But here we are presenting a case of cPAN who was admitied into Department of Dermatology and Venereology, Bangabandhu Sheikh Mujib Medical University with multiple sharply marginated hyper pigmented painful plaques over the left thigh with elevated vesicular margin and surrounding erythematous halo, without any subcutaneous nodules and with multiple pyoderma gangrenosum-like ulcers with necrotic slough with perilesional erythematous halo. Although only 20% of cPAN cases present without any subcutaneous nodules, and even fewer present with pyoderma gangrenosum like ulceration, this case report will reveal the rare possibility of presenting both and therefore will warrant the clinical suspicion of cPAN in case of lower limb ulcerations in a young lady. Keywords: Cutaneous Polyarteritis Nodosa(cPAN), subcutaneous nodules, Pyoderma Gangrenosum like ulcer in PAN. |