Linear nevus sebaceous syndrome
Other Names for this Disease
- Jadassohn nevus phakomatosis
- Nevus sebaceus of Jadassohn
- Organoid nevus phakomatosis
- Schimmelpenning Feuerstein Mims syndrome
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.
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). 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.
Linear nevus sebaceous syndrome is associated with vitamin D-resistant rickets due to secretions from the linear sebaceous nevus of Jadassohn (skin lesion).
The Human Phenotype Ontology provides the following list of signs and symptoms for Linear nevus sebaceous syndrome. If the information is available, the table below includes how often the symptom is seen in people with this condition. You can use the MedlinePlus Medical Dictionary to look up the definitions for these medical terms.
The Human Phenotype Ontology (HPO) has collected information on how often a sign or symptom occurs in a condition. Much of this information comes from Orphanet, a European rare disease database. The frequency of a sign or symptom is usually listed as a rough estimate of the percentage of patients who have that feature.
The frequency may also be listed as a fraction. The first number of the fraction is how many people had the symptom, and the second number is the total number of people who were examined in one study. For example, a frequency of 25/25 means that in a study of 25 people all patients were found to have that symptom. Because these frequencies are based on a specific study, the fractions may be different if another group of patients are examined.
Sometimes, no information on frequency is available. In these cases, the sign or symptom may be rare or common.
- 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.
- 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.
- 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.