[Intestinal malabsorption with a dissociated deficiency of immunoglobulins (author's transl)]

The case of a 42-year-old-man with dissociated deficiency of immunoglobulins and intestinal malabsorption is presented. The gastrointestinal symptoms included post-prandial pains and steatorrhea. Histologic and immunohistochemistry studies of small intestinal biopsies revealed subtotal villous atrophy and absence of IgA producing plasma cells. The deficiency of immunoglobulins was total for IgA and moderate for IgM. IgG was increased. HLA B8 haplotype was present Gluten-free diet gave dramatic improvement in few days on clinic symptoms. Recovery of the villi was observed within three months. The cases reported in the published literature are reviewed and the physiopathologic hypothesis and nosology limits of the syndrome are discussed.