Bullous ichthyosiform erythroderma (collodion baby; congenital ichthyosis; epidermolytic hyperkeratosis; ichthyosis; ichthyosis vulgaris; lamellar ichthyosis; nonbullous ichthyosiform erythroderma; xeroderma; x-linked ichthyosis) 182

General: Autosomal inherited disorder; affects both sexes; normal at birth; onset within first 7 days; X-linked; pathogenesis may be secondary to physicochemical changes of corneal tissues including accumulation of cholesterol sulfate.

Ocular: Keratopathy; corneal scarring; keratitis; conjunctivitis; lagophthalmos; photophobia; ectropion; lid erythema; lacrimation; keratoconus; deep corneal punctate/filiform lesions

Clinical: At birth, the skin surface is moist, red, and tender; within several days, thick verrucous scales form.

Casaroli Marano RP, et al. Ocular findings associated with congenital X-linked ichthyosis. Ann Ophthalmol 1991; 23:167-l72.

Fraunfelder FT, Roy FH. Current Ocular Therapy, 5th ed. Philadelphia: WB Saunders, 2000.

Frost P. Disorders of cornification. In: Moschella SL, et al., eds. Dermatology. Philadelphia: WB Saunders, 1975: 1056-l084.

Macsai MS, Doshi H. Clinical pathologic correlation of superficial corneal opacities in X-linked ichthyosis. Am J Ophthalmol 1994; 118:477-484.

Magalini SI, Scrascia E. Dictionary of Medical Syndromes, 2nd ed. Philadelphia: JB Lippincott, 1981.

Orth DH, et al. Collodion baby with transient bilateral lid ectropion. Review of ocular manifestations in ichthyosis. Arch Ophthalmol 1994; 91:206-207.