Korean J Dermatol.  2003 May;41(5):634-640.

A Case of Subcutaneous Panniculitic T-cell Lymphoma Involved in Skin and Central Nervous System

• Lee EJ
• Kim HW
• Seo YJ
• Lee JH
• Suhr KB
• Park JK
• Kim YC
• Cho KH

Abstract

Subcutaneous panniculitic T-cell lymphoma(SPTCL) is categorized as a rare subtype of peripheral T-cell lymphoma. It is characterized by primary involvement of the subcutaneous fat in a manner mimicking panniculitis with/without hemophagocytic syndrome. They share a generally aggressive course and are highly associated with Epstein-Barr virus infection. EBV infection plays an important role in the tumorigenesis and may be related to cutaneous lymphoma with hemophagocytic manifestations. We have seen a patient, a 67-year-old woman with tender erythematous nodules on both upper extremities and abdomen. With time, the skin lesion showed ulcerative change on her right thigh. She has also suffered from fever, weight loss, arthralgia, and general weakness without hepatosplenomegaly or lymphadenopathy for 4 months. During the admission, she complained of nausea, vomiting and dysarthria. On the MRI examination, we found a multi-focal solid lesion on her brain. The histopathological findings of the biopsy from her abdominal skin lesion showed a septal and lobular, histiocytic panniculitis with bean bag cells and atypical lymphoid cells identified as NK like T-cells and also dense diffuse infiltrates localized in the lower dermis and subcutaneous tissue, with minimal epidermal and upper dermal infiltrates without destructive change of blood vessels. The infiltrating atypical lymphoid cells expressed the phenotype of LCA, CD45RO, CD3, CD8, CD56 and also positive for EBV by in situ hybridization. Our case showed a clonal TCR gamma gene rearrangement by polymerase chain reaction. She was subjected to a course of treatment(cyclophosphamide, vincristine, prednisolone and radiation therapy) under the diagnosis of SPTCL. but died of sepsis due to urinary tract infections after 2 months.

Keyword

Subcutaneous panniculitic T-cell lymphoma; CNS involvement

MeSH Terms

Abdomen
Aged
Arthralgia
Biopsy
Blood Vessels
Brain
Carcinogenesis
Central Nervous System*
Dermis
Diagnosis
Dysarthria
Epstein-Barr Virus Infections
Female
Fever
Genes, T-Cell Receptor gamma
Herpesvirus 4, Human
Humans
In Situ Hybridization
Lymphatic Diseases
Lymphocytes
Lymphohistiocytosis, Hemophagocytic
Lymphoma
Lymphoma, T-Cell*
Lymphoma, T-Cell, Peripheral
Magnetic Resonance Imaging
Nausea
Panniculitis
Phenotype
Polymerase Chain Reaction
Prednisolone
Sepsis
Skin*
Subcutaneous Fat
Subcutaneous Tissue
T-Lymphocytes*
Thigh
Ulcer
Upper Extremity
Urinary Tract Infections
Vincristine
Vomiting
Weight Loss
Prednisolone
Vincristine