Scalded skin syndrome (toxic epidermal necrolysis; ritter disease; toxic epidermal necrolysis of lyell; staphylococcal scalded skin syndrome; lyell syndrome; epidermolysis acuta toxica; toxic epidermal necrolysis) 1

General: Generalized exfoliative dermatitis frequently affecting neonates and resulting from an initial focal staphylococcal infection (i.e., staphylococcal ophthalmia neonatorum); toxic epidermal necrolysis usually refers to manifestation in the adult secondary to a drug reaction but affects all ages; immunopathogenetic mechanisms probably initiated with drug-skin binding with aberrant immune responses, including complement and immunoglobulin G deposition with the epidermis and mucosa; recent reports suggest that patients with the acquired immunodeficiency syndrome (AIDS) are at higher risk for developing mucocutaneous reactions, such as toxic epidermal necrolysis; mortality rate approximately 30%.

Ocular: Necrotic areas of lids, conjunctiva, and cornea; symblepharon; loss of corneal epithelium; corneal ulcer; leukoma; perforation of globe; abolition of lacrimal secretion; conjunctival chemosis; blepharitis; entropion; periorbital swelling; trichiasis; distichiasis; fornix shortening.

Clinical: Widespread reddening and tenderness of the skin followed by the exfoliation of large areas of skin; in children, erythema starts usually around the mouth and spreads over the entire body within hours, followed by blisters and large exudative lesions; fever; shock.

Belfort R Jr, et al. Ocular complications of Stevens-Johnson syndrome and toxic epidermal necrolysis in patients with AIDS. Cornea 1991; 10:536-538.

Foster CS. Immunologic disorders of the conjunctiva, cornea, and sclera. In: Albert D, Jakobiec FA, eds. Principles and Practice of Ophthalmology, vol. I. Philadelphia: WB Saunders, 1994:190.

Kobayashi A, Yoshita T, Sugiyama K, et al. Amniotic membrane transtation in acute phase of toxic epidermal necrolysis with severe corneal involvement. Ophthalmology 2006; 113: 126-l32

Power WJ, et al. Analysis of the acute ophthalmic manifestations of the erythema multiforme/Stevens-Johnson syndrome/toxic epidermal necrolysis disease spectrum. Ophthalmology 1995:102:1669-l676.