Riley-Day Syndrome(CM Riley, R. L. Day - modern. Amer. Doctors) Riley-Day family autonomic dysfunction - a hereditary violation of the sympathetic transmission in the. n with. due to a defect in the formation or release of a mediator with an autosomal recessive mode of inheritance.
CLINICAL PICTURE. For patients with R.-D. with. Marble skin pattern, cold extremities, impaired thermoregulation, discordination of sucking and swallowing, hypersalivation, frequent constipation, decreased tendon and unconditioned reflexes, corneal reflex and pain sensitivity, mental lability are characteristic. Corneal ulceration appears due to the violation of tearing.
DIAGNOSIS is established on the basis of a characteristic clinical picture and a test with histamine (intradermal administration of histamine dihydrochloride in the form of a 0.1% solution is accompanied by the formation of a ring not surrounded by the normal erythema zone, in the absence of a pain reaction).
TREATMENT is symptomatic.