Juvenile unilesional folliculotropic mycosis fungoides: Two siblings with HLA-DRB1*04 and HLA-DQB1*03 alleles

Abstract

The pathogenesis of mycosis fungoides (MF) is poorly understood, and an immunogenetic mechanism has been suggested to play a role. Human leukocyte antigen (HLA) class II alleles DRB1*11 and DQB1*03 were found to be significantly increased for patients with sporadic and/ or familial MF. The juvenile-onset familial MF is extremely rare. Herein, we report two siblings diagnosed with folliculotropic MF, both exhibiting similar morphology of a unilesional presentation on the flexural region of the arms. Both were positive for HLA-DRB1*04 and HLA-DQB1*03 alleles. The HLA-DQB1*03 allele has been described in familial MF in the literature recently, whereas “HLA-DRB1*04” allele has not been reported previously in familial MF cases.

Keywords

• mycosis fungoides
• juvenile
• familial
• unilesional
• folliculotropic
• hla alleles