An unusual presentation of subcutaneous panniculitis-like T-cell lymphoma: Extensive necrosis and hemophagocytic lymphohistiocytosis: A case report.

KEY CLINICAL MESSAGE: Subcutaneous panniculitis-like T-cell lymphoma, a primary cutaneous lymphoma, which is described as following a slow course, could claim life. The occurrence of facial and breast nodules, the association with hemophagocytic lymphohistiocytosis, and the extent of necrosis and ulceration are signs of its aggressive nature needing early diagnosis and prompt treatment.

ABSTRACT: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare skin disease that accounts for <1% of all peripheral T-cell lymphomas. It is described as following a slow and gradual process. However, it can be associated with a variety of clinical symptoms ranging from mild to severe. Hemophagocytic lymphohistiocytosis (HLH), a rare and potentially fatal hematologic factor that complicates SPTCL in 20% of cases, is an important prognostic factor. We report here an aggressive case of disseminated SPTCL with HLH involving a young woman who presented with extensive necrosis and ulceration at diagnosis. The report highlights the aggressive course of the disease, the occurrence of facial and breast nodules, the association with HLH, and the extent of necrosis and ulceration. The report highlights the poor prognosis despite polychemotherapy regimen use.