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Babinski-Nageotte Syndrome to Burnett Syndrome -  

Bullous Ichthyosiform Erythroderma
Lecture 76 of 77  NEXT»

(Collodion Baby; Congenital Ichthyosis; Epidermolytic Hyperkeratosis; Ichthyosis; Ichthyosis Vulgaris; Lamellar Ichthyosis; Nonbullous Ichthyosiform Erythroderma; Xeroderma; X-Linked Ichthyosis)

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; 23:167-172.
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: 1056-1084.
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.


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