What Is Nevus of Ota?
Nevus of Ota (NOO-vuss) is a rare condition characterized by the inability to produce enough saliva. It affects only males and is caused by mutations in two genes. The most common form of NOO-vuss occurs when one or both copies of these genes are mutated, resulting in defective production of male hormones called androgens. These chemicals are essential for normal development and function of the body. Without them, male reproductive organs do not develop properly.
The affected individuals have no secondary sexual characteristics such as pubic hair, facial hair, testes or ovaries. They may still be able to reproduce normally if they receive fertility treatment. However, they will never be able to father children due to their inability to make sufficient amounts of testosterone.
In some cases, the mutation results in complete absence of any secondary sexual characteristics. Individuals with this type of NOO-vuss cannot reproduce either because they lack the ability to produce testosterone. They die before reaching adulthood.
How Is Nevus Of Ota Diagnosed?
A blood test to check for NOO-vuss can confirm a doctor’s suspicion. The results of this test can help the physician choose the most effective treatment plan.
What Are the Symptoms of Nevus Of Ota?
Symptoms of NOO-vuss include:
Poor growth and lack of secondary sexual characteristics such as facial hair, a deep voice and strong muscles
Small testicles, if present at all
What Causes Nevus Of Ota?
The cause is a defect in the genes that control the release of androgens by the body. The defect prevents the body from responding normally to androgens.
Who Gets Nevus Of Ota?
This condition is extremely rare. It has been identified in only two dozen families around the world. All of these families have a common ancestor. The defective gene, however, appears to also manifest itself in people who do not have NOO-vuss. The defect is just so slight that these individuals still produce enough androgens for proper development and function of their bodies.
How Is Nevus Of Ota Treated?
Treatment involves hormone supplements to increase androgen levels. The goal is to produce sufficient amounts of these hormones for normal development and function of the body. Hormone treatment may be required throughout the life of the patient and may be given in pill form, by injection or by a patch placed on the skin.
Can Nevus Of Ota Cause Complications?
The most common complication is infertility. However, some reports indicate that NOO-vuss can be a cause of cancer. A few studies have linked excess androgen exposure to an increased risk for certain types of cancers in both males and females.
What Is the Outlook for Nevus Of Ota?
The outlook is good for NOO-vuss patients who start hormone treatment. However, more long-term studies are needed before this can be stated with certainty.
What Is the Prognosis for Nevus Of Ota?
The Prognosis for NOO-vuss is excellent if the patient receives adequate hormone treatment.
Sources & references used in this article:
Treatment of nevus of Ota with the Q-switched ruby laser by S Watanabe, H Takahashi – New England Journal of Medicine, 1994 – Mass Medical Soc
Treatment of melanocytic nevi by H Suzuki, RR Anderson – Dermatologic therapy, 2005 – Wiley Online Library
Nevus of Ota with rare palatal involvement: A case report with emphasis on differential diagnosis by G Sharma, A Nagpal – Case reports in dentistry, 2011 – hindawi.com
Lifetime prevalence of uveal melanoma in white patients with oculo (dermal) melanocytosis by AD Singh, P De Potter, BA Fijal, CL Shields, JA Shields… – Ophthalmology, 1998 – Elsevier
Melanoma arising in blue nevus by G Massi, PE LeBoit – Histological Diagnosis of Nevi and Melanoma, 2014 – Springer
Sturge-Weber syndrome: management of glaucoma with combined trabeculotomy-trabeculectomy by HC Agarwal, S Sandramouli… – … Surgery, Lasers & …, 1993 – search.proquest.com
Uveal melanoma cell lines: where do they come from?(An American Ophthalmological Society Thesis) by MJ Jager, JAB Magner, BR Ksander… – Transactions of the …, 2016 – ncbi.nlm.nih.gov
Laser treatment for pigmented lesions: a review by CE Jones, K Nouri – Journal of cosmetic dermatology, 2006 – Wiley Online Library
Phakomatosis pigmentovascularis: clinical findings in 15 patients and review of the literature by MP Goldman, RE Fitzpatrick – Cutaneous Lasers Sur, Mosby Inc. St. Louis, 1999