Turkderm-Turk Arch Dermatol Venereol. 2022; 56(2): 76-79 | DOI: 10.4274/turkderm.galenos.2022.89083

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

Hatice Þanlý¹, Ýncilay Kalay Yýldýzhan¹, Bengü Nisa Akay¹, Canan Arý², Aylin Okçu Heper^3
1Ankara University Faculty of Medicine, Department of Dermatology, Ankara, Turkey
²University of Health Sciences Turkey, Dýþkapý Yýldýrým Beyazýt Training and Research Hospital, Clinic of Dermatology, Ankara, Turkey
³Ankara University Faculty of Medicine, Department of Pathology, Ankara,Turkey

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

---

Juvenil unilezyonel folikülotropik mikozis fungoides: HLA-DRB1*04 ve HLA-DQB1*03 allelleri ile iki kardeþ

Hatice Þanlý¹, Ýncilay Kalay Yýldýzhan¹, Bengü Nisa Akay¹, Canan Arý², Aylin Okçu Heper^3
1Ankara Üniversitesi Týp Fakültesi, Deri Ve Zührevi Hastalýklarý Ana Bilim Dalý, Ankara
²Dýþkapý Eðitim ve Araþtýrma Hastanesi, Dermatoloji Kliniði, Ankara
³Ankara Üniversitesi Týp Fakültesi, Patoloji Ana Bilim Dalý, Ankara

Mikozis fungoides (MF) patogenezi tam olarak anlaþýlamamýþtýr, immünogenetik mekanizmalarýn rol oynayabileceði öne sürülmüþtür. Ýnsan lökosit antijeni (HLA) sýnýf II allelleri DRB1*11 ve DQB1*03 sporadik ve/veya ailesel MF’si olan hastalarda önemli oranda yüksek bulunmuþtur. Juvenil baþlangýçlý ailesel MF ise oldukça nadirdir. Burada, her ikisinde de kollarýn fleksural bölgesinde tek taraflý benzer morfoloji sergileyen ve folikülotropik MF tanýsý alan iki kardeþi sunmaktayýz. Her iki kardeþde HLA-DRB1*04 ve HLA-DQB1*03 allelleri pozitif bulundu. HLA-DQB1*03 alleli literatürde yakýn zamanda ailesel MF’de tanýmlanmýþ olup, “HLA-DRB1*04” alleli daha önce ailesel MF olgularýnda bildirilmemiþtir.

Anahtar Kelimeler: Mikozis fungoides, juvenil, ailesel, unilezyonel, folikülotropik, HLA alelleri

---

CITE