Juvenile unilesional folliculotropic mycosis fungoides: Two siblings with HLA-DRB104 and HLA-DQB103 alleles/ Juvenil unilezyonel folikulotropik mikozis fungoides: HLA-DRB104 ve HLA-DQB103 allelleri ile iki kardes

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 Mikozis fungoides (MF) patogenezi tam olarak anlasilamamistir, immunogenetik mekanizmalarin rol oynayabilecegi one surulmustur. Insan lokosit antijeni (HLA) sinif II allelleri DRB1*11 ve DQB1*03 sporadik ve/veya ailesel MF'si olan hastalarda onemli oranda yuksek bulunmustur. Juvenil baslangicli ailesel MF ise oldukca nadirdir. Burada, her ikisinde de kollarin fleksural bolgesinde tek tarafli benzer morfoloji sergileyen ve folikulotropik MF tanisi alan iki kardesi sunmaktayiz. Her iki kardejde HLA-DRB1*04 ve HLA-DQB1*03 allelleri pozitif bulundu. HLA-DQB1*03 alleli literaturde yakin zamanda ailesel MF'de tanimlanmis olup, "HLA-DRB1*04" alleli daha once ailesel MF olgularinda bildirilmemistir. Anahtar Kelimeler: Mikozis fungoides, juvenil, ailesel, unilezyonel, folikulotropik, HLA alelleri