title: Lymphomatoid papulosis associated with mycosis fungoides: clinicopathological and molecular studies of 12 cases.
abstract: The association of mycosis fungoides and a primary cutaneous CD30+ lymphoproliferative disorder has been reported and probably represents different clinical aspects of a unique T-cell monoclonal expansion. In this study, 12 patients (6 men and 6 women) presented with lymphomatoid papulosis and mycosis fungoides. A TCRgamma gene rearrangement study was performed by an automated high-resolution PCR fragment analysis method on skin biopsy specimens taken from the different clinical lesions in each patient. An indolent clinical course was observed in the majority of patients. T-cell clonality was identified in 7 of 12 lymphomatoid papulosis lesions (58%) and in 6 skin biopsies of plaque stage mycosis fungoides (50%). In each individual case, where T-cell clonality was detected, both mycosis fungoides and lymphomatoid papulosis specimens exhibited an identical peak pattern by automated high-resolution PCR fragment analysis, confirming a common clonal origin. Only one case showed a clonal TCRgamma rearrangement from the lymphomatoid papulosis lesion, which could not be demonstrated in the mycosis fungoides specimen. The demonstration of an identical clone seems to confirm that both disorders are different clinical manifestations of a unique T-cell monoclonal proliferation. Our results also seem to confirm that the association of mycosis fungoides with a primary cutaneous CD30+ lymphoproliferative disorder usually carries a favourable prognosis.
authors.affiliation: Department of Dermatology and Pathology, Hospital del Mar, IMAS, Barcelona, Spain. FGallardo@imas.imim.es
authors.forename: Fernando
authors.initials: F
authors.lastname: Gallardo
authors.forename: Carlota
authors.initials: C
authors.lastname: Costa
authors.forename: Beatriz
authors.initials: B
authors.lastname: Bellosillo
authors.forename: Francesc
authors.initials: F
authors.lastname: Solé
authors.forename: Teresa
authors.initials: T
authors.lastname: Estrach
authors.forename: Octavio
authors.initials: O
authors.lastname: Servitje
authors.forename: María Pilar
authors.initials: MP
authors.lastname: García-Muret
authors.forename: Carlos
authors.initials: C
authors.lastname: Barranco
authors.forename: Sergi
authors.initials: S
authors.lastname: Serrano
authors.forename: Ramon M
authors.initials: RM
authors.lastname: Pujol
mesh_terms: D000328:Adult; D000368:Aged; D015703:Antigens, CD / Q000032:analysis; D001706:Biopsy; D002999:Clone Cells; D005260:Female; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor; D006801:Humans; D007150:Immunohistochemistry; D017731:Lymphomatoid Papulosis / Q000150:complications / Q000235:genetics / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009182:Mycosis Fungoides / Q000150:complications / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D016133:Polymerase Chain Reaction; D012867:Skin / Q000473:pathology; D012878:Skin Neoplasms / Q000150:complications / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D013601:T-Lymphocytes / Q000378:metabolism
chemical_list: D015703:Antigens, CD