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 (HPO) provides the following list of features that have been reported in people with this condition. Much of the information in the HPO comes from Orphanet, a European rare disease database. If available, the list includes a rough estimate of how common a feature is (its frequency). Frequencies are based on a specific study and may not be representative of all studies. You can use the MedlinePlus Medical Dictionary for definitions of the terms below.
|Signs and Symptoms||Approximate number of patients (when available)|
|Abnormal hair quantity||90%|
|Aplasia/Hypoplasia affecting the eye||90%|
|Aplasia/Hypoplasia of the cerebellum||90%|
|Vertebral segmentation defect||90%|
|Aplasia/Hypoplasia of the corpus callosum||7.5%|
|Abnormality of dental color||-|
|Abnormality of dental morphology||-|
|Abnormality of finger||-|
|Abnormality of toe||-|
|Basal cell carcinoma||-|
|Coarctation of aorta||-|
|Hypopigmentation of the skin||-|
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Living with a genetic or rare disease can impact the daily lives of patients and families. These resources can help families navigate various aspects of living with a rare disease.
These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.
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