Subcutaneous Panniculitis-Like T-Cell Lymphoma With Hemophagocytic Lymphohistiocytosis

Abstract

Subcutaneous panniculitis-like T-cell lymphoma (SPTLP), a unique variant of primary cutaneous T-cell lymphomas, clinically mimics subcutaneous panniculitis. It is typified by the development of multiple plaques or subcutaneous erythematous nodules, predominantly on the extremities and trunk. Epidemiological findings reveal a greater incidence in females than males, affecting a wide demographic, including pediatric and adult cohorts, with a median onset age of around 30 years. Diagnosis of SPTLP is complex, hinging on skin biopsy analyses and the identification of T-cell lineage-specific immunohistochemical markers. Treatment modalities for SPTLP are varied; while corticosteroids may be beneficial initially for many patients, a substantial number require chemotherapy, especially in cases of poor response or relapse. Generally, SPTLP progresses slowly, yet approximately 20% of cases advance to hemophagocytic lymphohistiocytosis (HLH), often correlating with a negative prognosis. We report a case of a young male patient presenting with prolonged fever, multiple skin lesions accompanied by HLH, a poor clinical course, and eventual death, diagnosed postmortem with SPTLP. In addition, we also present a literature review of the current evidence of some updates related to SPTLP.

Keywords: hemophagocytic lymphohistiocyosis; subcutaneous panniculitis-like T-cell lymphoma.

Figure 1.

Patient photographs showed lesions presenting as erythematous plaques and nodules (arrows) on the skin of the right lower leg (A) and abdomen (B).

Figure 2.

Microscopic images of hematoxylin-eosin staining sections: (A) Normal appearance of the epidermal and dermal layers (double-headed arrow, 200× magnification). (B) Subcutaneous fat layer infiltrated with numerous atypical lymphocytes (arrows), clustering around the periphery of fat cells (asterisks), with hyperchromatic nuclei, irregular nuclear membranes, and ranging in size from small to medium (400× magnification).

Figure 3.

Immunohistochemical staining images showed that tumor cells positive for CD3 (A, 400× magnification) and CD8 (B, 400× magnification), with stained brown-yellow; negative for CD4 (C, 400× magnification) and CD56 (D, 400× magnification).