Lupus erythematosus panniculitis: clinicopathological, immunophenotypic, and molecular studies.
Lupus erythematosus panniculitis (LEP) is an inflammatory disorder of the subcutaneous fat in patients with lupus erythematosus (LE). It is a rare variant of the disease, which occurs approximately in 1%-3% of patients with cutaneous LE. The purpose of this study was to investigate the clinical, histopathologic, immunophenotypical, and molecular profiles of LEP. We performed a retrospective study of 19 biopsy specimens from 17 patients with LEP. We reviewed their clinical data and reexamined the histopathology. Immunophenotyping and molecular studies were done using sections from paraffin-embedded formalin-fixed tissue. The most common clinical manifestation was a depressed patch on upper arm. Patients showed good response to variable treatment modalities, but, generally, relapse of panniculitis was noted when treatment was discontinued. Histopathologically, most specimens revealed lymphoplasmacytic lobular panniculitis with epidermal and dermal changes of LE, hyaline fat necrosis, and lymphoid follicles. Immunohistochemistry showed a mixture of T and B cells in dermis and subcutis with a slight preponderance of T cell. Although the polymerase chain reaction analysis of the T-cell receptor-gamma gene rearrangement showed a polyclonal smear in 89.5% of cases, a small portion of specimens demonstrated monoclonality. LEP is a chronic recurrent disease with characteristic features. Its diagnosis is often challenging, and a precise diagnosis is achievable only upon elaborate clinicopathologic correlation and integrated interpretation of all diagnostic criteria.
Park
Hyun Sun
HS
Department of Dermatology, Seoul National University College of Medicine, Seoul, Korea.
Choi
Jung Won
JW
Kim
Byoung-kwon
BK
Cho
Kwang Hyun
KH
D000293:Adolescent; D000328:Adult; D000368:Aged; D001402:B-Lymphocytes / Q000276:immunology / Q000473:pathology; D015415:Biomarkers / Q000378:metabolism; D002648:Child; D003622:Dapsone / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005218:Fat Necrosis / Q000276:immunology / Q000473:pathology; D005260:Female; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor / Q000235:genetics*; D006801:Humans; D006886:Hydroxychloroquine / Q000627:therapeutic use; D007124:Immunoenzyme Techniques; D016130:Immunophenotyping; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D008875:Middle Aged; D015435:Panniculitis, Lupus Erythematosus / Q000235:genetics / Q000276:immunology / Q000473:pathology; D011239:Prednisolone / Q000627:therapeutic use; D012189:Retrospective Studies; D012867:Skin / Q000276:immunology / Q000473:pathology; D013601:T-Lymphocytes / Q000276:immunology / Q000473:pathology; D055815:Young Adult
D015415:Biomarkers; D006886:Hydroxychloroquine; D003622:Dapsone; D011239:Prednisolone; D008727:Methotrexate