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Summary Xanthoma disseminatum (XD) is a rare benign histiocytic disorder with extensive mucocutaneous xanthomas that often involves other sites such as the central nervous system (CNS), respiratory tract and abdominal organs. Evaluation of the extent of disease is important because lesions in critical locations may increase morbidity and mortality. However, there are no well-established tools for the evaluation and monitoring of XD. Here, we report a case of XD in a 21-year-old male patient showing skin, mucous membrane, CNS and internal organ involvement. In this case, 18F-fluorodeoxyglucose positron emission tomography/computed tomography was useful in detecting the extent of the disease and in estimating the therapeutic response.

Nasal-type T/natural killer cell lymphoma (TNKCL) shows frequent extranodal involvement including the skin, and is associated with a poor prognosis. We have studied six patients with nasal-type TNKCL presenting with inflammatory subcutaneous nodular lesions with a subcutaneous lymphoid infiltrate. Clinical information was obtained from the medical records of the patients and at follow-up. All cases showed features of angiocentric lymphoma on histology, although there was diffuse cellular infiltration rather than an angiocentric pattern in the subcutis. All six patients were similar in immunophenotype: positive for CD56 and either cytoplasmic CD3 or CD45RO, but negative for B-cell markers and CD30. In situ hybridization using an anti-sense Epstein-Barr virus early regions probe showed a positive reaction in all cases. All patients either died with progressive disease or showed no response to combined chemotherapy. The diagnosis of nasal-type TNKCL, which has a fatal outcome, is facilitated by detection of CD56-positive tumour cells. In evaluating lobular panniculitis including apparently benign inflammatory subcutaneous nodules, nasal-type TNKCL should be considered in the differential diagnosis, especially in Asian countries.

We describe clinicopathological features of an unusual case of CD30+/CD56+ T-cell lymphoma in a 58-year-old Korean man who presented with disseminated nodules, papules and hyperpigmented patches. Coexpression of CD30 and CD56 in T-cell lymphoma is very rare. Our patient did not respond to an intensive chemotherapy regimen, in contrast to the previously reported cases of primary cutaneous CD30+ anaplastic large cell lymphoma. Coexpression of CD56 might therefore identify a subset of CD30+ lymphomas with more aggressive features.