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A 37-year-old lady presented to our out patient department with a history of low-grade fever and multiple painful erythematous nodules on the body, predominantly distributed on the extremities of 2 months duration. Within 3 days, they progressed to form deep punched out ulcers [Figure 1]. She was also found to have atrophic rhinitis. She gave no history of photosensitivity, malar rash, morning stiffness, or trauma. She has no history of any drug intake prior to the appearance of the lesions. She had no motor or sensory deficits.

Figure 1.

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Necrotic punched ulcers

Based on the clinical findings, the initial differential diagnoses considered included Pyoderma gangrenosum, Wegeners granulomatosis, and nodular vasculitis. On investigating the patient, she was found to have leukocytosis with a total count of 15000/cumm. The other routine laboratory investigations, Mantoux intradermal test, autoantibody screening, viral and serological analysis, blood culture, and swabs for bacteria were unremarkable.

Histopathology of the skin revealed a large intradermal cleft filled with fibrin and a few inflammatory cells. The epidermis overlying the cleft has orthokeratosis, parakeratosis, hypergranulosis, and acanthosis. The dermis showed dilated vessels with endothelial cells and fibrinoid degeneration of the vessel wall surrounded by neutrophilic dust. A dense granulomatous infiltrate with lymphocytes and macrophages was found around the vessel walls [Figure 2a]. Septal and lobular panniculitis was present [Figure 2b]. Taking the clinical picture into consideration, a diagnosis of nodular vasculitis was made, and treatment was instituted for the same with oral steroids and dapsone. But, despite institution of therapy, the response to treatment was unsatisfactory.