DERMATOLOGICAL MANIFESTATIONS OF FAMILIAL AUTONOMIC DYSFUNCTION (RILEY-DAY SYNDROME)

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Abstract
A CURSORY review of dermatologic literature indicates that the Riley-Day syndrome is not known. In the past a number of children have been seen by hospital services and private physicians presenting "symptoms so puzzling as to defy exact diagnosis yet so similar as to constitute a clinical entity.''1 This syndrome is characterized by the following more or less constant features: (1) Jewish extraction, (2) defective lacrimation, (3) skin blotching, (4) excessive perspiration, (5) drooling, (6) emotional instability, (7) motor incoordination, (8) hyporeflexia, (9) relative indifference to pain, (10) hypertension, (11) cyclic vomiting, (12) frequent pulmonary infection, (13) frequent unexplained fever, (14) breath-holding spells in infancy, (15) urinary frequency, (16) mental retardation, (17) convulsions, and (18) corneal ulcerations. In August, 1952, Riley reported 33 cases,2 in 10 of which the patients died, and in which autopsy had been performed on nine. Riley proposed the term "familial autonomic

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