Nodular vasculitis as a paraneoplastic presentation

A previously healthy 71-year-old Caucasian woman presented with an ulcer on her left leg. She had a 5-month history of bilateral, indurated erythematous, and had purplish nodules and plaques on both lower extremities. One of the plaques on the right leg had ulcerated and healed spontaneously. The present ulceration began as a small opening at an incisional biopsy site. Later, she developed two adjacent ulcers that coalesced into a larger ulcer. She was not taking any medication, and there was no history of exposure to tuberculosis. Examination of the right leg demonstrated two hyperpigmented, indurated plaques, one on the calf and one over the medial malleolus. On the left posterior calf there was another hyperpigmented, indurated plaque with a central area of ulceration measuring 9 × 2 cm. The ulcer bed had a mixture of fibrinous adherent tissue and granulation tissue. (Fig. 1) There was no peripheral edema, varicose veins, nor signs of peripheral neuropathy. Peripheral pulses and capillary refilling were within normal limits. The ankle brachial index was greater than 1.0 bilaterally. Biopsy of a subcutaneous nodule revealed a superficial and deep perivascular lymphocytic infiltrate with an admixture of neutrophils and numerous extravasated red blood cells. Mixed septal and lobular panniculitis consisting of an inflammatory infiltrate of histiocytes, giant cells, and neutrophils was observed. Focal neutrophilic microabscesses in the deep dermis and fibrinoid necrosis of small blood vessels were present in the deep dermis and subcutaneous tissue (Figs 2 and 3). A smear was negative for acid fast bacilli. Figure 1. A hyperpigmented, indurated plaque with a central area of ulceration measuring 9 × 2 cm on the left posterior calf. The ulcer bed has a mixture of fibrinous adherent tissue and granulation tissue Download figure to PowerPoint Figure 2. A scanning magnification Download figure to PowerPoint Figure 3. Mixed septal and lobular panniculitis consisting of an inflammatory infiltrate of histiocytes, giant cells, and neutrophils. Focal neutrophilic microabscesses in the deep dermis and fibrinoid necrosis of small blood vessels in the deep dermis and subcutaneous tissue Download figure to PowerPoint A diagnosis of nodular vasculitis was made. A complete blood count with differential and platelets was within normal limits, tuberculin testing was negative twice, and antineutrophil cytoplasmic antibodies (p-ANCA, c-ANCA) were also negative. Fungal, mycobacterial and bacterial cultures were negative. Chest X-rays were normal. The patient received supersaturated potassium iodide (SSKI) 15 drops per day with good response. With rapid tapering of SSKI, the ulcerations on the lower legs reappeared. Therapy was reinstituted with a more gradual taper. Two years after the initial diagnosis of nodular vasculitis the patient presented with new complaints of increased urinary frequency, increased abdominal girth, and weight gain. The patient was found to be borderline anemic, and a computer tomography scan revealed ascites, a normal liver, a normal uterus, and a soft tissue density in the region of the cecum. After an exploratory laparotomy she was found to have a moderately differentiated mucinous adenocarcinoma of the colon with metastases to periaortic lymph nodes, abdominal wall, and both ovaries. She is currently receiving adjuvant chemotherapy for stage IV colon carcinoma. Only one lesion of nodular vasculitis has remained active, and the patient is continuing treatment with SSKI at a low dose.