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Rajsmita Bhattacharjee, Debajyoti Chatterjee, Tarun Narang, Sunil Dogra, Necrotic erythema nodosum leprosum masquerading as cutaneous vasculitis, Rheumatology, Volume 58, Issue 1, January 2019, Page 85, https://doi.org/10.1093/rheumatology/key216
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A young male in his 30s presented with fever, joint pains, multiple tender nodules and ulcerated skin lesions of 2 months duration. Multiple crusted ulcers with sharply defined borders and surrounding rim of erythema mimicking cutaneous vasculitis were present on the trunk and extremities (Fig. 1A and B). Cutaneous examination revealed madarosis with diffuse infiltration of the skin of face and ears (Fig. 1C). Ulnar, radial cutaneous and common peroneal nerves (right > left) were asymmetrically thickened with glove and stocking anaesthesia. Slit skin smear showed multiple acid-fast bacilli. Histopathological examination from the noduloulcerative lesions confirmed necrotic erythema nodosum leprosum (ENL) (Fig. 1D). A diagnosis of previously undiagnosed lepromatous leprosy presenting with necrotic ENL was rendered.
ENL, an immune complex-mediated type 2 leprosy reaction, is seen most commonly in the lepromatous spectrum of Hansen’s disease. It classically presents as crops of tender, evanescent nodules usually involving the extremities, trunk and face, and associated with systemic symptoms like fever, malaise, joint pains and lymphadenopathy. Apart from the classical lesions, other less common but severe morphological variants of ENL include vesicobullous, pustular and necrotic types. Necrotic ENL can often mimic cutaneous vasculitis. Hence, dermatologists and clinicians should be aware of the different presentations of this disease for prompt diagnosis and treatment thereby avoiding debilitating consequences. A high degree of suspicion and close attention to the often subtle ancillary skin findings are imperative, especially in areas where leprosy is still widely prevalent.
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