Primary Cutaneous T-Cell Lymphoma Localized to the Lower Leg

Abstract

Background: Distinct categories of skin lymphoma with preferential site localization and unique clinical behavior, including leg-type primary cutaneous diffuse large B-cell lymphoma, have recently been described. Although these entities are rare, they exhibit reproducible clinicopathologic features, and their recognition may allow more appropriate treatment protocols.Observations: We describe the distinctive clinicopathologic features that were observed in 3 patients with an unusual variant of primary cutaneous T-cell lymphoma. All cases originated on the legs of elderly patients and exhibited a locally aggressive clinical behavior with relatively rapid relapses after radiotherapy and resistance to other therapies. Histologically, dense dermal-centered infiltrates of atypical, variably sized mature helper T cells were identified. One patient died of progressive disease.Conclusions: Rare cases of primary cutaneous lymphomas do not necessarily fit current criteria for a standard diagnostic category but may represent unique clinicopathologic entities, such as primary cutaneous T-cell lymphoma localized to the lower leg. It is important to be able to identify these unusual lymphoma variants for prognosis and adequate treatment. The aggressive nature of lymphomas preferentially localized on the lower extremities may not be restricted to B-cell or cytotoxic neoplasms.

Cutaneous manifestations of peripheral T‐cell lymphoma, not otherwise specified: A case series highlighting the diagnostic challenges for this heterogeneous group

“…6 More recently, an atypical presentation of a faint erythematous eruption was described by a group from Japan. 7 Our series was similar to larger collaborative studies in many findings, including male predominance, mean age of 71.2 years, duration between skin manifestations and diagnosis, treatment choice of systemic anthracyclinecontaining chemotherapy for late stage disease, and poor prognosis.…”

Primary Cutaneous Anaplastic Large Cell Lymphoma—A Review of Clinical, Morphological, Immunohistochemical, and Molecular Features

“…In general, primary cutaneous ALCL has a favorable prognosis with a 10-year overall survival rate of 90% [ 15 ]. However, studies have shown that patients with lesions located in the upper and lower extremities have a worse prognosis [ 16 , 17 ]. Primary cutaneous ALCL can affect immunosuppressed individuals, including those with human immunodeficiency virus (HIV) infection, status post organ transplantation, or those treated with immunomodulatory drugs, such as adalimumab (tumor necrosis factor inhibitor), which is used to treat patients with autoimmune disorders, or fingolimod, which is used to treat patients with relapsing–remitting multiple sclerosis [ 17 , 18 , 19 ].…”