Denouement and Discussion
Epidermolytic hyperkeratosis, previously designated as bullous congenital ichthyosiform erythroderma, is a rare form of ichthyosis. Manifestations are usually present at birth. The epidermis is thick and macerated, resembling a wet blotter. Portions of skin may have already been desquamated in sheets, leaving raw, denuded, weeping patches. These findings are characteristic of a burned baby rather than a colloidion baby. The thick, macerated membrane rapidly sheds, leaving a raw, moist, tender surface that may resemble the skin present in other diseases in which blistering is present. Bullae are the most characteristic manifestation of the disease, and their presence differentiates epidermolytic hyperkeratosis from the other types of ichthyosis. The recurrent bullae usually last throughout childhood, become less prominent with time, and may resolve in early adolescence. Occasionally, bullae may be present in adults. The face is frequently less severely affected than other areas of the body.
Jones BE, Mehrabani D, Engelke S, Feingold M. Picture of the Month. Am J Dis Child. 1988;142(11):1231-1232. doi:10.1001/archpedi.1988.02150110109031