INTRAVASCULAR LYMPHOMATOSIS (ANGIOTROPIC LARGE-CELL LYMPHOMA), A RARECAUSE OF PAINFUL SWELLING OF THE LEG WITH TREATMENT-RESISTANT FEVER

History and clinical findings: For one month a 69-year-old woman had b een suffering from increasingly painful and reddened swelling of both legs and induration of the skin of the left thigh, about 15 cm in diam eter. In addition she had fever and rigors. Antibiotic treatment, begu n because erysipelas was suspected, was ineffective and she was hospit alized. Although obese she was in a good general condition with no obv ious abnormalities on routine lung, heart and neurological examination . No lymph nodes were palpated. Investigations: Laboratory tests showe d increased inflammatory parameters, marked rise in lactate dehydrogen ase and a normochromic anaemia, hemoglobin of 9.5 g/dl. Doppler sonogr aphy excluded deep vein thrombosis, but marked chronic venous insuffic iency was revealed. Extensive tests, including soft-tissue sonography, radiology and skin biopsy failed to establish a diagnosis. Treatment and cause: Antibiotic treatment was resumed because a diagnosis of onl y partly treated erysipelas was made. But several changes to a variety of antibiotics remained ineffective. Collagen disease was excluded by the biochemical and biopsy results. As the skin changes in both legs increased a skin and muscle biopsy from the indurated area of the left thigh was done: it showed intravascular large-cell lymphomatosis. A s earch for a paraneoplastic process revealed an adenocarcinoma of the a scending colon that was successfully resected. The patient died before the planned chemotherapy could be initiated. Conclusion: In case of t reatment-resistant fever associated with painful swelling of the leg a nd skin changes of unknown etiology a deep skin biopsy should be an ea rly consideration to exclude such causes as lymphoma, including the ve ry rare intravascular clear-cell lymphomatosis.