Granulomatous Slack Skin: Clonal Rearrangement of the T-Cell Receptor β Gene Is Evidence for the Lymphoproliferative Nature of a Cutaneous Elastolytic Disorder

Granulomatous slack skin (GSS) is characterized by the slow evolution of bulky, erythematous skin folds that have a granulomatous histology, and show destruction of dermal elastic tissue. Several cases have been putatively associated with Hodgkin's disease, and histologic similarities to mycosi...

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Veröffentlicht in:Journal of investigative dermatology 1987-08, Vol.89 (2), p.183-186
Hauptverfasser: Leboit, Philip E., Beckstead, Jay H., Bond, Barbara, Epstein, William L., Frieden, Ilona J., Parslow, Tristrarn G.
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container_end_page 186
container_issue 2
container_start_page 183
container_title Journal of investigative dermatology
container_volume 89
creator Leboit, Philip E.
Beckstead, Jay H.
Bond, Barbara
Epstein, William L.
Frieden, Ilona J.
Parslow, Tristrarn G.
description Granulomatous slack skin (GSS) is characterized by the slow evolution of bulky, erythematous skin folds that have a granulomatous histology, and show destruction of dermal elastic tissue. Several cases have been putatively associated with Hodgkin's disease, and histologic similarities to mycosis fungoides have also been noted. We examined tissue from 3 cases of GSS to determine whether the condition was inflammatory or lymphoproliferative in nature. We found an abnormal, monomorphous T-helper cell immunophenotype, and in all 3 cases, Clonal rearrangement of the T-cell receptor β gene. We conclude that GSS is an indolent cutaneous T-cell lymphoma associated with granulomatous inflammation that mediates elastolysis, producing a distinctive clinical appearance.
doi_str_mv 10.1111/1523-1747.ep12470557
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subjects Dermatology
Evolution
Female
gene rearrangement
Genes
Granuloma - immunology
Granuloma - pathology
Hodgkin's disease
Humans
Inflammation
Lymphocytes
Lymphoproliferative Disorders - immunology
Lymphoproliferative Disorders - pathology
Male
Mycosis fungoides
Nucleic Acid Hybridization
Phenotype
Receptors, Antigen, T-Cell - genetics
Skin
Skin - immunology
Skin - pathology
Skin Diseases - immunology
Skin Diseases - pathology
T-cell lymphoma
T-cell receptor
T-Lymphocytes - immunology
title Granulomatous Slack Skin: Clonal Rearrangement of the T-Cell Receptor β Gene Is Evidence for the Lymphoproliferative Nature of a Cutaneous Elastolytic Disorder
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