Overview
Nevus of Ota, also known as oculodermal melanocytosis or nevus fuscocaeruleus ophthalmomaxillaris, is a benign melanocytic condition characterized by blue-gray to blue-brown hyperpigmentation affecting the skin in the distribution of the first and second branches of the trigeminal nerve (ophthalmic and maxillary divisions). The condition results from the presence of ectopic dermal melanocytes that become trapped in the upper dermis during embryonic migration. It most commonly affects the periorbital area, temple, forehead, malar region, and nose on one side of the face, though bilateral involvement occurs in a small percentage of cases. The sclera (white of the eye) is frequently involved, giving the eye a bluish or slate-gray discoloration, and pigmentation may also extend to the conjunctiva, cornea, retina, tympanic membrane, nasal mucosa, and palate. Nevus of Ota is most prevalent among individuals of Asian descent, particularly Japanese populations, and is also seen in individuals of African and Hispanic ancestry. It is significantly more common in females than males. The condition typically presents at birth or during early childhood, though a second peak of onset occurs around puberty, likely influenced by hormonal changes. While Nevus of Ota is generally a benign condition, there is a small but recognized risk of malignant transformation to melanoma, particularly uveal melanoma or cutaneous melanoma arising within the nevus. Patients with ocular involvement should undergo regular ophthalmologic examinations to monitor for glaucoma, which is an associated complication. Treatment is primarily pursued for cosmetic reasons and to reduce the theoretical risk of malignant transformation. Q-switched laser therapy (including Q-switched ruby, alexandrite, and Nd:YAG lasers) is the current standard of care and has demonstrated excellent results in lightening or clearing the pigmentation with minimal scarring. Multiple treatment sessions are typically required. Camouflage cosmetics may also be used. Ongoing surveillance by a dermatologist and ophthalmologist is recommended for early detection of any complications, including melanoma and glaucoma.
Also known as:
Sporadic
Usually appears on its own, not inherited from a parent
Variable
Can begin at different ages, from infancy through adulthood
Treatments
No FDA-approved treatments are currently listed for Nevus of Ota.
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Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Nevus of Ota.
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Common questions about Nevus of Ota
What is Nevus of Ota?
Nevus of Ota, also known as oculodermal melanocytosis or nevus fuscocaeruleus ophthalmomaxillaris, is a benign melanocytic condition characterized by blue-gray to blue-brown hyperpigmentation affecting the skin in the distribution of the first and second branches of the trigeminal nerve (ophthalmic and maxillary divisions). The condition results from the presence of ectopic dermal melanocytes that become trapped in the upper dermis during embryonic migration. It most commonly affects the periorbital area, temple, forehead, malar region, and nose on one side of the face, though bilateral involv
How is Nevus of Ota inherited?
Nevus of Ota follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
Which specialists treat Nevus of Ota?
2 specialists and care centers treating Nevus of Ota are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.