August 1970

Picture of the Month

Author Affiliations

Boston; Kansas City, Kan
From the Department of Pediatrics, University of Kansas Medical Center, Kansas City, Kan.

Am J Dis Child. 1970;120(2):139-140. doi:10.1001/archpedi.1970.02100070083009

Denouement and Discussion 

Linear Nevus Sebaceous Syndrome 

Manifestations  The major manifestations include sebaceous nevi, seizures, and mental retardation. The skin lesions are present at birth and are located on the forehead and extend to the tip of the nose. They may also be found on the face and body where they present a linear configuration. Other skin findings include pigmented nevi and hirsutism. There is a variety of abnormalities of the skull including hydrocephalus, asymmetry, premature closure of the sphenofrontal suture, and widening of the sella turcica. Abnormalities of the eyes include antimongoloid slant, nystagmus, lipodermoids, scarring and vascularization of the cornea, choroidal coloboma, and blindness. The bridge of the nose is broadened. Oral findings include hypoplastic teeth and thickened mucosa secondary to nevi which may be present on the hard and soft palate. Coarctation and hypoplasia of the aorta have been reported. These children usually fail to thrive.

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