Psychological aspects of hypohidrotic ectodermal dysplasia.

There is little information on the psychologic aspects of HED. In particular, there is insufficient evidence to support or dispute the claim that the intelligence of persons with HED is dissimilar to that of the general population. Impressions of frequent mental retardation may arise from patients suffering brain damage following high fever and from our biases against the physically unattractive. A small number of adults with HED have been reported to hold a relatively wide range of jobs, and an even smaller number have been reported with educational and occupational achievement that is predictive of bright average to superior intellectual functioning, but even less evidence is available here. Chronic illness and handicaps exert a toll on the patient and the family. There appears to be greater maladjustment among the families of chronically ill children, but this differs according to the type of illness, and we do not know how this affects the HED child and his/her family. Still, the HED family is exposed to some unique stresses: having always to take into account the ambient temperature regardless of their planned activity; responding to cruel stares and remarks when out in public; and dealing with the problem of broken, lost, or merely publicly discovered dentures. On the other hand, some children apparently benefit from having a sib with a disability. Families react in a variety of ways to a disability, and their reaction may interact with the child's temperament to affect emotional development for better or worse. However, the existence of HED does not insure either normal or inadequate emotional adjustment, and parents should be advised of this. As difficult as it may be, this may also be the time for the pediatrician to say "I don't know." Parents can be told that each child, not just those with HED, should be provided a warm and supportive environment congruent with his/her unique style, in order to facilitate emotional development. They should also be advised of the probable emotional benefits of early prosthetic care with wigs and dentures. When this is either not possible or not sufficient, professional guidance should be sought. Psychotherapy or counseling can be obtained for the child, a parent, or for the entire family. While some familiarity with HED may be helpful, it is not essential, and is far less important than finding a competent professional with whom the family is comfortable. As far as obtaining the information we need, a series of systematic investigations appears to be called for.(ABSTRACT TRUNCATED AT 400 WORDS)