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February 1959

Aldrich's Syndrome (Thrombocytopenia, Eczema, and Infection in Infants): Studies of the Defense Mechanisms

Author Affiliations


AMA Am J Dis Child. 1959;97(2):137-153. doi:10.1001/archpedi.1959.02070010139001

Aldrich, Steinberg, and Campbell,1 in 1954, described a sex-linked recessive condition in infants which was characterized by draining ears, eczematoid dermatitis, and bloody diarrhea. Huntely and Dees,2 and Wolff and Bertucio3 reported 11 additional infants and children who have had the unusual triad of severe eczema, thrombocytopenia, and early death resulting from overwhelming infection. This disease appeared to be a definite entity which, although uncommon, is not extremely rare.

Because of the distressing morbidity and unfavorable prognosis despite all current therapy and because the etiology of this interesting syndrome remains an enigma, continued investigation of this disease was considered necessary.

It is the purpose of the present paper to report seven additional instances of this syndrome in which extensive morphological, immunological, hematological, endocrinological, and clinical investigations were completed.

Report of Cases  Case 1.—This child was admitted to the University of Minnesota Hospitals at the age of 3

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