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Linear nevus sebaceus of jadassohn (nevus sebaceus of jadassohn; jadassohn-type anetoderma; organoid nevus syndrome; sebaceus nevus syndrome )


Linear Nevus Sebaceus of Jadassohn (Nevus Sebaceus of Jadassohn; Jadassohn-Type Anetoderma;
Organoid Nevus syndrome; Sebaceus nevus syndrome ) 131j94b 131j94b 131j94b 131j94b

General: Skin nevus caused by failure of separation of skin appendages from adjacent epithelium during the third month of gestation.

Ocular: Proptosis; epibulbar lipodermoids; colobomata of eyelids, iris, and choroid; antimongoloid fissures; ocular motor palsies; nystagmus; teratomas of orbit and aberrant lacrimal glands; corneal vascularization; vision defects; conjunctival dermolipomas; choristomas of conjunctiva, sclera; corneal vascularization/opacification; colobomas of uvea, retina, optic disk, and lids; optic nerve hypoplasia; microphthalmia; anophthalmia; hemangioma of the sclera/conjunctiva.

Clinical: Circumscribed lesions of the face and scalp with excessively large sebaceous glands; papillomatous epidermal hyperplasia; seizures; skeletal abnormalities, particularly in skull; failure to thrive; convulsion; mental retardation.

Brenner S, et al. Jadassohn-type anetoderma m association with keratoconus and cataract. Ophthalmologica 1977; 174:181-l84.

Garella M, Ghosh B, Thakar M, et al. Linear nevus sebaceous syndrome with bilateral complex limbal choristomas and extraocular muscle involvement. Ann Ophthalmol 2004; 36: 59-62

Haslam RHA, Wirtschafler JD. Unilateral external oculomotor nerve palsy and nevus sebaceus of Jadassohn. Arch Ophthalmol 1972; 87:293.

Jadassohn J. Bemerkungen fur Histolgre der System Atisirten Naeviund uber 'Talgdrusen-Naevi.' Arch Dermatol Syphil 1895; 33:355.

Katz B, et al. Optic nerve hypoplasia and the syndrome of nevus sebaceous of Jadassohn. Ophthalmology 1987; 94: 1570.

Wilkes SR, et al. Ocular malformation in association with ipsilateral facial nevus of Jadassohn. Am J Ophthalmol 1981; 92:344-352.




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