Epidermolytic hyperkeratosis

Synonym(s)

Bullous congenital ichthyosiform erythro-derma; bullous ichthyotic erythroderma;

ichthyosis bullosa of Siemens; ichthyosis hystrix of Curth-Macklin

Definition

Congenital ichthyosis with characteristic histologic finding of epidermolytic hyperk-eratosis

Pathogenesis

Autosomal dominant trait; defect in the genes for keratin 1 and keratin 10

Clinical manifestation

Presents at birth or shortly thereafter as erythema, blistering, and/or scaling; marked hyperkeratosis shortly after birth; scales are small, dark, with corrugated appearance; scales sometimes shedand, reaccumulate; keratotic skin in intertrigi-nous areas which may become macerated and foul smelling; blisters occur in crops, rupturing, and leaving red, painful, denuded base; bullae tend to disappear before age 20; NPS subtype - lacks severe palmoplantar involvement; PS subtype -severe palmoplantar involvement; no ectro-pion

Differential diagnosis

Non-bullous ichthyosiform erythroderma; lamellar ichthyosis; X-linked ichthyosis; epidermolysis bullosa; incontinentia pig-menti; bullous impetigo; staphylococcal scalded skin syndrome

Therapy

Prednisone; beta carotene; acetretin; tretin-oin; alpha-hydroxy acid

References

Bale SJ, Compton JG, DiGiovanna JJ (1993) Epi-dermolytic hyperkeratosis. Seminars in Dermatology 12(3):202-209

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