Bullous congenital ichthyosiform erythroderma (BCIE)

Definition:

Bullous congenital ichthyosiform erythroderma (BCIE), also known as epidermolytic hyperkeratosis (EHK), is characterized by erythema and skin blistering of the newborn. The erythema is replaced with thick scaling later. Neonates with BCIE have higher risk of developing severe infection, such as sepsis.

References:

[1]. Dawn H Siegel, et al. Molecular advances in genetic skin diseases. Curr Opin Pediatr. 2002 Aug;14(4):419-25. [Content Brief]

[2]. Nicole L Lacz, et al. Epidermolytic hyperkeratosis: a keratin 1 or 10 mutational event. Int J Dermatol. 2005 Jan;44(1):1-6. [Content Brief]

[3]. Rustin Ross, et al. Histopathologic characterization of epidermolytic hyperkeratosis: a systematic review of histology from the National Registry for Ichthyosis and Related Skin Disorders. J Am Acad Dermatol. 2008 Jul;59(1):86-90. [Content Brief]

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