title: Mycosis fungoides in childhood: description and study of two siblings.
abstract: Primary cutaneous T-cell lymphomas are exceedingly rare in children and adolescents. However, mycosis fungoides (MF) is the most frequent primary cutaneous lymphoma diagnosed in childhood. Two cases of MF in siblings (a 14-year-old boy and his 10-year-old sister) are reported. On the basis of clinical features (histopathological and immunophenotypical findings) a diagnosis of MF patch lesions was made in both siblings. Since recent data in the literature have underlined a high frequency of the HLA-DQB1*03 allele in patients with familial MF (including child patients), the HLA profile of the patients was analysed, indicating the presence of a haplotype (HLA-DQB1*03,*03 in the girl, HLA-DQB1*02,*03 in the boy) corresponding with that described in recent literature. Two rare and exceptional cases of MF in siblings are reported, highlighting the presence of a peculiar haplotype.
authors.affiliation: Department of Dermatology, University of Pavia, Pavia, Italy. cvassallo@yahoo.com
authors.forename: Camilla
authors.initials: C
authors.lastname: Vassallo
authors.forename: Valeria
authors.initials: V
authors.lastname: Brazzelli
authors.forename: Enza
authors.initials: E
authors.lastname: Cestone
authors.forename: Michela
authors.initials: M
authors.lastname: Castello
authors.forename: Olga
authors.initials: O
authors.lastname: Ciocca
authors.forename: Riccardo Giovanni
authors.initials: RG
authors.lastname: Borroni
authors.forename: Miryam
authors.initials: M
authors.lastname: Martinetti
authors.forename: Giovanni
authors.initials: G
authors.lastname: Borroni
mesh_terms: D000293:Adolescent; D015703:Antigens, CD / Q000032:analysis; D002648:Child; D005260:Female; D006683:HLA-DQ Antigens / Q000235:genetics*; D059866:HLA-DQ beta-Chains; D006684:HLA-DR Antigens / Q000235:genetics; D059811:HLA-DRB1 Chains; D006239:Haplotypes; D006801:Humans; D008297:Male; D009182:Mycosis Fungoides / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy; D035781:Siblings; D012867:Skin / Q000473:pathology; D013601:T-Lymphocytes / Q000276:immunology; D014467:Ultraviolet Therapy
chemical_list: D015703:Antigens, CD; D006683:HLA-DQ Antigens; D059866:HLA-DQ beta-Chains; C069051:HLA-DQB1 antigen; D006684:HLA-DR Antigens; D059811:HLA-DRB1 Chains