A Fatal Granulomatous Disease of Childhood:  The Clinical, Pathological, and Laboratory Features of a New Syndrome

In 1950, a 12-month-old child was referred to the University of Minnesota Hospitals with a syndrome consisting of chronic suppurative lymphadenitis, hepatosplenomegaly, pulmonary infiltrations, and an eczematoid dermatitis about the eyes, nose, and mouth. Since then we have seen three other children with an almost identical clinical picture. This syndrome appears to be a distinct clinical entity not previously described in the medical literature. This syndrome follows a malignant course and at this time has been responsible for the death of all four of these patients. In the management of these patients we have utilized a wide variety of regimens. No satisfactory therapy has been found, however, and the disease has relentlessly progressed through severe debilitation to ultimate death over a period of several years. The clinical syndrome presented by these patients is similar to various disease states that have been previously well characterized. The most striking similarity is with