Subcutaneous Panniculitis-like T Cell Lymphoma Mimicking Early-Onset Nodular Panniculitis

Abstract

Patient: Male, 24Final Diagnosis: Subcutaneous panniculitis-like T-cell lymphomaSymptoms: Fever • skin nodulesMedication: —Clinical Procedure: Skin biopsy • PET-CTSpecialty: HematologyObjective:Rare diseaseBackground:Subcutaneous panniculitis-like T cell lymphoma is a very uncommon subtype of cutaneous T cell lymphoma. The manifestations of this rare disease are atypical at onset, and may mimic some rheumatic or dermatologic diseases, which causes the delay of diagnosis and treatment.Case Report:We report a 24-year-old man suffering from intermittent fever and skin nodules on the left anterior chest wall, who was initially misdiagnosed with nodular panniculitis and finally diagnosed with subcutaneous panniculitis-like T cell lymphoma through repeat examination of biopsy of the skin nodule. Positron emission tomography revealed extracutaneous adipose tissue involvement. Subsequently, hemophagocytic syndrome occurred while under a conventional dose of glucocorticoid, but remission was induced by treatment with cyclosporine A and high doses of dexamethasone.Conclusions:In order to avoid the delay diagnosis and inappropriate treatment of subcutaneous panniculitis-like T cell lymphoma, in addition to a thorough physical examination, PET-CT and disease-specific pathologic, immunophenotypic, and T cell receptor tests of the skin biopsy should be performed. Extracutaneous involvement, especially hemophagocytic syndrome, indicated worse prognosis. Even so, cyclosporine A plus high-dose corticosteroid could be an option of treatment.

Clinical characteristics, differential diagnosis, and treatment outcome of subcutaneous panniculitis-like T-cell lymphoma: a literature review of published Japanese cases

“…In our cases associated with HPS, none responded to conventional-dose chemotherapy. These results are in accordance with the previous view that CHOP or CHOP-like therapy is not very successful for SPTCL associated with HPS [4], and methylprednisolone pulse therapy, either alone or in combination with immunosuppressive drugs, can be effective for patients associated with HPS [33][34][35]. In addition, highdose chemotherapy, followed by stem cell transplantation, has been suggested to be an important option for patients associated with HPS [4,36,37].…”

Hemophagocytic Lymphohistiocytosis in Cutaneous T-Cell Lymphoma

“…Although cyclosporine is generally contraindicated in most CTCL types, it has been successful in some cases of HLH and CTCL and may be a viable option in this context. [9][10][11][12][13] In our experience, cyclosporine helped to manage the initial HLH stage but was not successful as a long-term option for the malignant condition. Because controlling HLH and CTCL may require different therapies, treatment combinations may be required to achieve long-lasting responses.…”

“…Given the rarity of HLH and CTCL, no established treatment guidelines exist for patients with both conditions. Although cyclosporine is generally contraindicated in most CTCL types, it has been successful in some cases of HLH and CTCL and may be a viable option in this context . In our experience, cyclosporine helped to manage the initial HLH stage but was not successful as a long-term option for the malignant condition.…”

Subcutaneous Panniculitis-like T-Cell Lymphoma with a Transformation to Lupus Erythematosus Panniculitis: A Case Report

“…Treatment options are categorized into immunosuppressives (corticosteroids alone or in combination with ciclosporin A or methotrexate) and chemotherapy (cyclophosphamide, doxorubicin, vincristine, and prednisone) [ 11 , 35 , 36 ]. Several studies demonstrated cases with SPTCL achieved complete remission after given ciclosporin A alone or combined with steroids as the first-line therapy [ 37 , 38 , 39 , 40 , 41 , 42 , 43 ]. Our patient achieved response after given the therapy.…”