Contributor: Harry H. Brown
Jadassohn nevus phakomatosis (linear sebaceous nevus syndrome, linear nevus sebaceous of Jadassohn, organoid nevus syndrome, Solomon syndrome, epidermal nevus syndrome, Schimmelpenning syndrome, Schimmelpenning-Feuerstein-Mims syndrome) is a multisystem syndrome with manifestations that involve the skin, central nervous system, eyes and other structures and usually occurs sporadically. It consists of raised congenital nevi of sebaceous glands. which often emit a foul odor. The numerous ophthalmic manifestations include epibulbar dermoids [dermoid] and choristomas [choristoma]. In this phakomatosis multiple small, irregular shaped, hairless papules with a yellowish orange or tan color are present at birth on the body, face, neck and scalp. Sometimes the oral mucosa is affected. Over time the papules change their appearance and become verrucous and nodular by adolescence. When the scalp is involved in the nevus the affected area is bald. The nevi are often large and do not extend across the midline. Often a large nevus extends from the forehead to the tip of the nose.