Abstract

<h3>To the Editor.—</h3> Since Navaratnam and Hodgson<sup>1</sup>described the first patient in 1973, there have been 24 reported cases of the cardiofaciocutaneous (CFC) syndrome, a genetic disorder of unknown cause with characteristic facies, cardiac defects, mental retardation, and cutaneous abnormalities. The cause of this disorder is presently unknown, and, although a genetic defect has been suggested, its exact pattern of inheritance is unclear. We describe a young boy with CFC syndrome who, in addition to the typical multisystem features, demonstrated café au lait lesions, acanthosis nigricans, and a syringocystadenoma papilliferum. <h3>Report of a Case.—</h3> Our patient, an 8-year-old boy born to unrelated Italian parents, was admitted to The Johns Hopkins Hospital, Baltimore, Md, for evaluation of severe mental retardation and self-injurious behavior. He had the characteristic facies of the CFC syndrome, including bitemporal constriction, hypertelorism, epicanthal folds, severe ptosis, a flattened nasal bridge, and low-set and posteriorly rotated ears

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