[Intravascular lymphomatosis (angiotrophic large-cell lymphoma), a rare differential diagnosis in painful swelling of the leg with treatment-resistant fever].

HISTORY AND CLINICAL FINDINGS: For one month a 69-year-old woman had been suffering from increasingly painful and reddened swelling of both legs and induration of the skin of the left thigh, about 15 cm in diameter. In addition she had fever and rigors. Antibiotic treatment, begun because erysipelas was suspected, was ineffective and she was hospitalized. Although obese she was in a good general condition with no obvious abnormalities on routine lung, heart and neurological examination. No lymph nodes were palpated.

INVESTIGATIONS: Laboratory tests showed increased inflammatory parameters, marked rise in lactate dehydrogenase and a normochromic anaemia, hemoglobin of 9.5 g/dl. Doppler sonography excluded deep vein thrombosis, but marked chronic venous insufficiency 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 only 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 search for a paraneoplastic process revealed an adenocarcinoma of the ascending colon that was successfully resected. The patient died before the planned chemotherapy could be initiated.

CONCLUSION

In case of treatment-resistant fever associated with painful swelling of the leg and skin changes of unknown etiology a deep skin biopsy should be an early consideration to exclude such causes as lymphoma, including the very rare intravascular clear-cell lymphomatosis.