Darier-White Syndrome (Keratosis Follicularis;
Dyskeratosis Follicularis Syndrome;
Psorospermosis)& 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; & 323b15d nbsp; 345
General: Unknown etiology; defect in the synthesis, organization, and maturation of tonofilament-desmosome complex; irregular dominant inheritance; both sexes equally affected, with onset in childhood; chronic but relatively benign and more aggravated in the summer.
Ocular: Conjunctival keratosis; bilateral corneal subepithelial infiltrations and sometimes corneal ulceration; cataract formation (rare).
Clinical: Confluent flesh-colored keratotic papules on head, neck, back, abdomen, and groin; small stature; mild mental retardation; hair loss; genital hypoplasia; oral-mucosal lesions; hypertrophic flexural involvement; acral signs.
Blackman HJ, et al. Corneal epithelial lesions in keratosis follicularis (Darier's disease). Ophthalmology 931-943.
Burge SM, Wilkinson JD. Darier- White disease: a review of the Clinical features in 163 patients. J Am Acad Dermatol 1992; 27:40-50.
Darier J. Psorospermatose folliculaire vegetante. Ann Dermatol Syphil 1889; 10:597.
Geeraets WJ. Ocular Syndromes, 3rd ed. Philadelphia: Lea & Febiger, 1976.
Magalini SI, Scrascia E. Dictionary of Medical Syndromes, 2nd ed. Philadelphia: JB Lippincott, 1981.
Mohamed KN. Darier's disease and corneal opacity. Dermatologica 1991; 182:205.
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