Aldrich's Syndrome (Thrombocytopenia, Eczema, and Infection in Infants)

Aldrich, Steinberg, and Campbell,1in 1954, described a sex-linked recessive condition in infants which was characterized by draining ears, eczematoid dermatitis, and bloody diarrhea. Huntely and Dees,2and Wolff and Bertucio3reported 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 Case1.—This child was admitted to the University of Minnesota Hospitals at the age of 3