Annular epidermolytic ichthyosis.

A 21-year-old woman presented to the Charles C. Harris Skin and Cancer Pavilion with a life-long history of palmoplantar keratoderma that was accompanied by episodic flares of polycyclic psoriasiform patches diffusely over the rest of her body. A biopsy specimen of a representative skin lesion showed epidermolytic hyperkeratosis. The particular phenotype that this patient exhibited has been reported in only seven families previously in the literature. Mutations in keratin 1 and 10 have been identified in these patients and are similar to those mutations in patients with classic epidermolytic hyperkeratosis.