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


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ŞANLI H., YILDIZHAN İ., AKAY B. N., Ari C., HEPER A.

TURKDERM-TURKISH ARCHIVES OF DERMATOLOGY AND VENEROLOGY, cilt.56, sa.2, ss.76-79, 2022 (ESCI) identifier

Özet

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.