A Rare Case of Subcutaneous Panniculitis-Like T Cell Lymphoma with Hemophagocytic Lymphohistiocytosis Mimicking Cellulitis

Abstract

Patient: Male, 48-year-old Final Diagnosis: Hemophagocytic Lymphohistiocytosis • subcutaneous panniculitis-like T cell lymphoma Symptoms: Chills • erythema • fever • night sweats • subcutaneous nodules Medication: — Clinical Procedure: Autologous stem cell transplantation • chemotherapy Specialty: Dermatology • Hematology • Infectious Diseases • Medicine, General and Internal • Oncology • Pathology • Transplantology Objective: Rare disease Background: Subcutaneous panniculitis-like T cell lymphoma and primary cutaneous γδ T cell lymphoma are rare forms of non-Hodgkin lymphoma presenting as skin nodules or plaques. Case Report: Here, we present a case of a 48-year-old man with multiple subcutaneous, tender, erythematous nodules on his right thigh and left arm. Multiple courses of antibiotics were administered with no significant improvement in the patient’s lesions. The skin biopsy report showed CD3/CD8 lymphocytic rimming of the adipocytes and the patient was diagnosed with subcutaneous panniculitis-like T cell lymphoma. A subsequent bone marrow biopsy showed hemophagocytic lymphohistiocytosis. The patient underwent treatment with the cyclophosphamide, hydroxydaunorubicin, Oncovin, prednisone, and etoposide chemotherapy regimen and received an autologous peripheral blood stem cell transplant. Conclusions: Nodular skin lesions can result from a variety of noninfectious causes in addition to bacterial and fungal infections. This case highlights the importance of early biopsy of skin lesions that do not respond to standard therapy to establish an accurate diagnosis and start timely treatment to prevent poor outcomes.

A unique case of subcutaneous panniculitis-like T-cell lymphoma presenting as an abscess following an arthropod bite

“…The initial presentation of SPTCL can mimic pyoderma and patients can present with features such as fever, erythema, and tender swelling. However, these persist despite courses of antibiotics with bacterial cultures returning sterile [2,10]. To the best of our knowledge, aside from the case reported herein, there are two other reports exemplifying how pseudo-pyoderma may be a pitfall in the diagnosis of SPTCL.…”

“…Subcutaneous panniculitis-like T-cell lymphoma commonly presents as multiple non-tender subcutaneous nodules which are localized on the trunk and limbs [1][2][3]. In addition to our case, various benign-looking, non-characteristic histological appearances have been encountered in SPTCL, including that of lipomembraneous panniculitis [5].…”

“…Based on the WHO-EORTC classification, SPTCL is a diagnosis reserved for cases with the ɑ/β T-cell phenotype that are CD4-, CD8+, and CD56- [1][2][3]. It is a rare form of cutaneous lymphoma with good prognosis, generally affecting young adults [4].…”

“…A distinction should be made from cases of cutaneous ɣ/δ T-cell lymphoma involving the subcutaneous compartment as these are conversely associated with rapid clinical deterioration and poorer prognosis. Although both entities may exhibit expression of granzyme B, TIA-1 and perforin, the latter are usually CD4-, CD8-, and CD56+ [1][2][3].…”

“…Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare form of cutaneous lymphoma involving infiltration of cytotoxic T cells localized to the subcutaneous tissue with infrequent lymph node involvement. Resembling a panniculitis, it commonly presents as multiple non-tender subcutaneous nodules localized on the trunk and limbs [1][2][3]. Herein, we present an unusual patient with SPTCL presenting as an abscess.…”