THIRTEEN BOYS WITH PROGRESSIVE SEPTIC GRANULOMATOSIS

Abstract

1. Thirteen boys are described who present a syndrome of chronic suppurative lymphadenitis, chronic dermatitis, chronic pulmonary disease, and hepatosplenomegaly, with an eventually fatal outcome. 2. The cases described appear to have the same basic disorder as those previously reported by Landing and Shirkey and by Bridges, Berendes, and Good. 3. Despite the frequent finding of hypergammaglobulinemia, no defect of antibody synthesis has been demonstrated. 4. The existence of an as yet unidentified physiological defect common to these patients is suggested.