Among four patients with positive anti-DGP and/or anti-tTG, only one had been previously diagnosed with CD and IgG subclass deficiency, one was under immunological care due to AN and lymphocyte T deficiency with a history of pyogenic infections, two were complaining of chronic diarrhoea (one of them had selective IgA deficiency and the other IgM deficiency). The gene discussed is TGM2; the disease is Nezelof syndrome.