Granulomatous slack skin: clonal rearrangement of the T-cell receptor beta 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 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 beta gene. We conclude that GSS is an indolent cutaneous T-cell lymphoma associated with granulomatous inflammation that mediates elastolysis, producing a distinctive clinical appearance.
D005260:Female; D005796:Genes; D006099:Granuloma / Q000276:immunology* / Q000473:pathology; D006801:Humans; D008232:Lymphoproliferative Disorders / Q000276:immunology* / Q000473:pathology; D008297:Male; D009693:Nucleic Acid Hybridization; D010641:Phenotype; D011948:Receptors, Antigen, T-Cell / Q000235:genetics*; D012867:Skin / Q000276:immunology* / Q000473:pathology; D012871:Skin Diseases / Q000276:immunology* / Q000473:pathology; D013601:T-Lymphocytes / Q000276:immunology*
D011948:Receptors, Antigen, T-Cell