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Subcutaneous Panniculitis‐Like T‐Cell Lymphoma in Children: A Review of the Literature
Author(s) -
Hu Zhi Li,
Sang Hong,
Deng Lin,
Li ZongHui
Publication year - 2015
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/pde.12452
Subject(s) - panniculitis , medicine , pathology , gene rearrangement , lymphoma , cutaneous lymphoma , histopathology , t cell lymphoma , vincristine , prednisolone , skin biopsy , biopsy , mycosis fungoides , cyclophosphamide , chemotherapy , biology , gene , biochemistry
Subcutaneous panniculitis‐like T‐cell lymphoma ( SPTCL ) is a type of skin lymphoma. Pleomorphic T‐cells infiltrate the subcutaneous tissue and mimic lobular panniculitis. We report a case occurring in a 12‐year‐old Chinese boy who presented with multiple indolent erythematous subcutaneous nodules on both extremities without systemic symptoms. He had a protracted course of multiple erythematous subcutaneous nodules for 1 year and underwent biopsy of lesional skin for histology and T‐cell receptor ( TCR ) gene analysis. Histopathology showed infiltration of medium to large atypical pleomorphic cells involving the subcutis with characteristic rimming of fat spaces. TCR gene rearrangement shows monoclonal rearrangements of the TCR β and γ chains. Immunophenotypic studies showed that CD 3, CD 4, and CD 8 were strongly and diffusely positive in lesional cells and that CD 56 was focally positive. In contrast, these cells were negative for CD 20, CD 30, and CD 68. The combined morphology, characteristic histologic features, and positive T‐CR gene rearrangement supported a diagnosis of SPTCL . He is being treated with combination chemotherapy of cyclophosphamide, doxorubicin, vincristine, and prednisolone.