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.[2]
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).[2] 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.[4]
Linear nevus sebaceous syndrome is associated with vitamin D-resistant rickets due to secretions from the linear sebaceous nevus of Jadassohn (skin lesion).[4]
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% |
| Adenoma sebaceum | 90% |
| Aplasia/Hypoplasia affecting the eye | 90% |
| Aplasia/Hypoplasia of the cerebellum | 90% |
| Asymmetric growth | 90% |
| Cavernous hemangioma | 90% |
| Cognitive impairment | 90% |
| EEG abnormality | 90% |
| Frontal bossing | 90% |
| Genu recurvatum | 90% |
| Hyperreflexia | 90% |
| Iris coloboma | 90% |
| Melanocytic nevus | 90% |
| Muscular hypotonia | 90% |
| Narrow forehead | 90% |
| Prominent occiput | 90% |
| Seizures | 90% |
| Telecanthus | 90% |
| Vertebral segmentation defect | 90% |
| Facial asymmetry | 50% |
| Irregular hyperpigmentation | 50% |
| Plagiocephaly | 50% |
| Porencephaly | 50% |
| Aplasia/Hypoplasia of the corpus callosum | 7.5% |
| Cerebral calcification | 7.5% |
| Dandy-Walker malformation | 7.5% |
| Corneal opacity | 5% |
| Hyperphosphaturia | 5% |
| Ophthalmoplegia | 5% |
| Precocious puberty | 5% |
| Abnormality of dental color | - |
| Abnormality of dental morphology | - |
| Abnormality of finger | - |
| Abnormality of toe | - |
| Alopecia | - |
| Basal cell carcinoma | - |
| Coarctation of aorta | - |
| Coloboma | - |
| Cranial asymmetry | - |
| Hemangioma | - |
| Hemimegalencephaly | - |
| Horseshoe kidney | - |
| Hypophosphatemic rickets | - |
| Hypopigmentation of the skin | - |
| Ichthyosis | - |
| Intellectual disability | - |
| Kyphoscoliosis | - |
| Nevus sebaceous | - |
| Osteopenia | - |
| Overgrowth | - |
| Recurrent fractures | - |
| Short stature | - |
| Somatic mosaicism | - |
| Sporadic | - |
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At what age does a child get symptoms of sebaceous nevus? See answer
I was wondering if you could provide me with more information on linear sebaceous nevus syndrome. My son was diagnosed with this and I am having a real hard time finding information on it. See answer
