Polysaccharide specific humoral immunodeficiency and ectodermal dysplasia - Case report of a male patient with two affected brothers

We report a now three year old male patient with ectodermal dysplasia and a polysaccharide specific humoral immunodeficiency. Immunological investigat ions showed compromised production of IgA, IgM, and IgG(2). Isohaemagglutin ins still were not detectable at the age of three years. Repeated vaccinati on with polpvalent pneumococcal polysaccharide vaccine did not result in pr oduction of specific antibodies. Two brothers showed clinical signs of ecto dermal dysplasia. The elder brother died from pneumococcal sepsis at the ag e of 3 years. The younger brother suffers from chronic inflammatory gastroi ntestinal disease with ulcerations in all parts of the gastrointestinal sys tem. Thus, a possible association between polysaccharide specific humoral i mmunodeficiency and ectodermal dysplasia map be considered.