A neurologist should assess and manage seizures. Dermatologists may help confirm the diagnosis by skin biopsy and may be able to help with cosmetic improvement of scars. Ear, nose, and throat surgeons can assess involvement of the upper airway and may be able to remove vocal cord nodules if present. A geneticist should review the patient's and parents' family history and provide counseling regarding the risks of having further affected children. Retinoids - Retinoic acid analogs that modulate cell growth and proliferation.
Mechanism of action is unknown.
Laryngeal involvement may lead to respiratory obstruction and voice change. Cerebral involvement is believed to be the cause of seizures and behavioral change in these patients. Small bowel deposition of hyaline has been reported to cause gastrointestinal bleeding. Lipoid proteinosis runs a stable or slowly progressive course. It is compatible with a normal life span.