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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Linear nevus sebaceous syndrome


Other Names for this Disease
  • Jadassohn nevus phakomatosis
  • JNP
  • Nevus sebaceus of Jadassohn
  • Organoid nevus phakomatosis
  • Schimmelpenning Feuerstein Mims syndrome
More Names
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Symptoms


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What are the signs and symptoms of linear nevus sebaceous syndrome?

Symptoms of linear nevus sebaceous syndrome include linear sebaceous nevus of Jadassohn, hemimegalencephaly (abnormal enlargment of one side of the brain), seizure, intellectual disability,[1][2] eye abnormalities, skeletal deformities, cancer, and heart defects.[3]

Linear nevus sebaceus of Jadassohn is characterized by single or multiple skin lesions. The lesions can form extensive hairless plaques, slightly elevated, circumscribed, with a soft velvety surface, yellowish in white individuals and hyperpigmented in black individuals. During puberty, the lesions grow and become more evident, warty, and sometimes nodular. During adulthood, patients have an increased risk for developing skin tumors within the lesion.[3]

Eye symptoms may include abnormally small eyes, lipodermoid scleral tumors (yellowish-white, fatty, non-cancerous scleral tumors), corneal opacity (cloudiness of the front part of the eye), eye calcifications, and coloboma (a structural defect of the eye).[3] Click here to view an interactive diagram of the eye.

People with linear nevus sebaceous syndrome may be at an increased risk for developing certain cancers such as skin, breast, salivary gland, stomach, ameloblastoma, esophageal, and bladder cancer.[2]

Linear nevus sebaceous syndrome is associated with vitamin D-resistant rickets due to secretions from the linear sebaceous nevus of Jadassohn (skin lesion).[2]

Some patients with linear nevus sebaceous syndrome have heart defects, such as coarctation of the aorta and ventricular septal defects.[2]
Last updated: 8/22/2011

References
  1. Lien SH, Hsu ML, Yuh YS, Lee CM, Chen CC, Chang PY, Chou CY. Prenatal three dimensional ultrasound detection of linear nevus sebaceous syndrome. Archives of Disease in Childhood Fetal and Neonatal Edition. 2005; http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1721920/pdf/v090p0F315.pdf. Accessed 7/21/2011.
  2. Herman TE, Siegel MJ. Journal of Perinatology. 2001; http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=DetailsSearch&Term=11536031%5Buid%5D. Accessed 5/6/2008.
  3. Terenzi V, Indrizzi E, Buonaccorsi S, Leonardi A, Pellacchia V, Fini G.. Nevus sebaceus of Jadassohn. J Craniofac Surg. 2006; http://www.ncbi.nlm.nih.gov/pubmed/17119437. Accessed 7/21/2011.