No special diet is needed. No activity restrictions are necessary. Neonatal patients may need to be transferred to the NICU for monitoring of infection, sepsis, electrolyte imbalance, and administration of intravenous fluids or antibiotics. Schedule routine follow-up visits as needed for symptomatic relief or to follow laboratory studies during systemic therapies. Transfer of infants to the NICU to monitor for sepsis and electrolyte imbalance may be necessary.
Patients are at an increased risk for recurrent infections, and a pungent smell can be noted.
Some patients may experience amelioration of symptoms as they age. Educate patients concerning the potential of passing the chromosomal defect on to offspring. Genetic approaches to understanding the keratinopathies. Mutations in the genes for epidermal keratins in epidermolysis bullosa and epidermolytic hyperkeratosis. Prenatal diagnosis for keratin mutations to exclude transmission of epidermolytic hyperkeratosis.