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 |
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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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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.</description><identifier>ISSN: 0022-202X</identifier><identifier>EISSN: 1523-1747</identifier><identifier>DOI: 10.1111/1523-1747.ep12470557</identifier><identifier>PMID: 3496402</identifier><language>eng</language><publisher>United States: Elsevier Inc</publisher><subject>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</subject><ispartof>Journal of investigative dermatology, 1987-08, Vol.89 (2), p.183-186</ispartof><rights>1987 The Society for Investigative Dermatology, Inc</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c418t-bd11c5b3a9cd2f9d73a3a9676cdf1876bce7fa32c926096dc8296f257dcead743</citedby><cites>FETCH-LOGICAL-c418t-bd11c5b3a9cd2f9d73a3a9676cdf1876bce7fa32c926096dc8296f257dcead743</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,776,780,27901,27902</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/3496402$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Leboit, Philip E.</creatorcontrib><creatorcontrib>Beckstead, Jay H.</creatorcontrib><creatorcontrib>Bond, Barbara</creatorcontrib><creatorcontrib>Epstein, William L.</creatorcontrib><creatorcontrib>Frieden, Ilona J.</creatorcontrib><creatorcontrib>Parslow, Tristrarn G.</creatorcontrib><title>Granulomatous Slack Skin: Clonal Rearrangement of the T-Cell Receptor β Gene Is Evidence for the Lymphoproliferative Nature of a Cutaneous Elastolytic Disorder</title><title>Journal of investigative dermatology</title><addtitle>J Invest Dermatol</addtitle><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.</description><subject>Dermatology</subject><subject>Evolution</subject><subject>Female</subject><subject>gene rearrangement</subject><subject>Genes</subject><subject>Granuloma - immunology</subject><subject>Granuloma - pathology</subject><subject>Hodgkin's disease</subject><subject>Humans</subject><subject>Inflammation</subject><subject>Lymphocytes</subject><subject>Lymphoproliferative Disorders - immunology</subject><subject>Lymphoproliferative Disorders - pathology</subject><subject>Male</subject><subject>Mycosis fungoides</subject><subject>Nucleic Acid Hybridization</subject><subject>Phenotype</subject><subject>Receptors, Antigen, T-Cell - genetics</subject><subject>Skin</subject><subject>Skin - immunology</subject><subject>Skin - pathology</subject><subject>Skin Diseases - immunology</subject><subject>Skin Diseases - pathology</subject><subject>T-cell lymphoma</subject><subject>T-cell receptor</subject><subject>T-Lymphocytes - immunology</subject><issn>0022-202X</issn><issn>1523-1747</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1987</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kcFu1DAQhi0EKkvhDUDyCU4pthPbCQckFJal0gokWiRulteeUNMkTm1npX0bnoEH4ZlwtKtyqy-2Zr75Zzw_Qi8puaD5vKWclQWVlbyAibJKEs7lI7S6Dz9GK0IYKxhhP56iZzH-IoSKitdn6KysGlERtkK_N0GPc-8Hnfwc8VWvzS2-unXjO9z2ftQ9_gY6ZOYnDDAm7DucbgBfFy30S87AlHzAf__gDYyALyNe752F0QDucnxht4dhuvFT8L3rIOjk9oC_6DQHWNQ0buekR1i6r3sdk-8PyRn80UUfLITn6Emn-wgvTvc5-v5pfd1-LrZfN5fth21hKlqnYmcpNXxX6sZY1jVWljq_hRTGdrSWYmdAdrpkpmGCNMKamjWiY1xaA9rKqjxHb466edC7GWJSg4smf_I4m2pETXnTCJ7J1w-SUvKaE04yWB1BE3yMATo1BTfocFCUqMVCtXilFq_Ufwtz2auT_rwbwN4XnTzL-ffHPOR17B0EFY1bNm5dAJOU9e7hBv8A-n-u0A</recordid><startdate>19870801</startdate><enddate>19870801</enddate><creator>Leboit, Philip E.</creator><creator>Beckstead, Jay H.</creator><creator>Bond, Barbara</creator><creator>Epstein, William L.</creator><creator>Frieden, Ilona J.</creator><creator>Parslow, Tristrarn G.</creator><general>Elsevier Inc</general><scope>6I.</scope><scope>AAFTH</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><scope>7T5</scope><scope>8FD</scope><scope>FR3</scope><scope>H94</scope><scope>P64</scope><scope>RC3</scope></search><sort><creationdate>19870801</creationdate><title>Granulomatous Slack Skin: Clonal Rearrangement of the T-Cell Receptor β Gene Is Evidence for the Lymphoproliferative Nature of a Cutaneous Elastolytic Disorder</title><author>Leboit, Philip E. ; Beckstead, Jay H. ; Bond, Barbara ; Epstein, William L. ; Frieden, Ilona J. ; Parslow, Tristrarn G.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c418t-bd11c5b3a9cd2f9d73a3a9676cdf1876bce7fa32c926096dc8296f257dcead743</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1987</creationdate><topic>Dermatology</topic><topic>Evolution</topic><topic>Female</topic><topic>gene rearrangement</topic><topic>Genes</topic><topic>Granuloma - immunology</topic><topic>Granuloma - pathology</topic><topic>Hodgkin's disease</topic><topic>Humans</topic><topic>Inflammation</topic><topic>Lymphocytes</topic><topic>Lymphoproliferative Disorders - immunology</topic><topic>Lymphoproliferative Disorders - pathology</topic><topic>Male</topic><topic>Mycosis fungoides</topic><topic>Nucleic Acid Hybridization</topic><topic>Phenotype</topic><topic>Receptors, Antigen, T-Cell - genetics</topic><topic>Skin</topic><topic>Skin - immunology</topic><topic>Skin - pathology</topic><topic>Skin Diseases - immunology</topic><topic>Skin Diseases - pathology</topic><topic>T-cell lymphoma</topic><topic>T-cell receptor</topic><topic>T-Lymphocytes - immunology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Leboit, Philip E.</creatorcontrib><creatorcontrib>Beckstead, Jay H.</creatorcontrib><creatorcontrib>Bond, Barbara</creatorcontrib><creatorcontrib>Epstein, William L.</creatorcontrib><creatorcontrib>Frieden, Ilona J.</creatorcontrib><creatorcontrib>Parslow, Tristrarn G.</creatorcontrib><collection>ScienceDirect Open Access Titles</collection><collection>Elsevier:ScienceDirect:Open Access</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><collection>Immunology Abstracts</collection><collection>Technology Research Database</collection><collection>Engineering Research Database</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>Biotechnology and BioEngineering Abstracts</collection><collection>Genetics Abstracts</collection><jtitle>Journal of investigative dermatology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Leboit, Philip E.</au><au>Beckstead, Jay H.</au><au>Bond, Barbara</au><au>Epstein, William L.</au><au>Frieden, Ilona J.</au><au>Parslow, Tristrarn G.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Granulomatous Slack Skin: Clonal Rearrangement of the T-Cell Receptor β Gene Is Evidence for the Lymphoproliferative Nature of a Cutaneous Elastolytic Disorder</atitle><jtitle>Journal of investigative dermatology</jtitle><addtitle>J Invest Dermatol</addtitle><date>1987-08-01</date><risdate>1987</risdate><volume>89</volume><issue>2</issue><spage>183</spage><epage>186</epage><pages>183-186</pages><issn>0022-202X</issn><eissn>1523-1747</eissn><abstract>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.</abstract><cop>United States</cop><pub>Elsevier Inc</pub><pmid>3496402</pmid><doi>10.1111/1523-1747.ep12470557</doi><tpages>4</tpages><oa>free_for_read</oa></addata></record> |
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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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