Folliculotropic mycosis fungoides associated withGATA2deficiency: a new skin manifestation

Abstract

DEAR EDITOR, MonoMAC syndrome, or GATA2 deficiency, is an autosomal dominant immune disease. It is characterized by monocytopenia; B-cell, natural-killer-cell 1 and CD4 + T-cell lymphopenia; nontuberculous mycobacterial or viral infections; lymphoedema; and myeloid malignancies [myelodysplastic syndrome (MDS) and acute myeloid leukaemia]. 2 GATA2 mutations can also cause vascular, ear or neurological manifestations, and neoplasia. Recently, Polat et al. reviewed skin manifestations associated with GATA2 deficiency. 3 We report for the first time a case of mycosis fungoides (MF) in a patient with MonoMAC syndrome.A 28-year-old man presented with repeated bacterial folliculitis, scrotal lymphoedema, recurrent lung infections, complicated cystic pulmonary parenchymal disorders, and refractory pancytopenia (MDS without excess of blasts). He also had congenital anacusia. Biological tests showed monocytopenia (60 9