Establishing the diagnosis
- Congenital blue nevus of periocular skin
- Tumor of melanocytes in the upper third of the dermis
- Dermal melanocytes proliferate in the region of ophthalmic (V1) and maxillary (V2) dermatomes of the trigeminal nerve.
- Typically on one side of the face
- When patchy slate gray pigmentation also occurs on the episclera and uvea, as occurs in two-thirds of affected patients, the disease is known as oculodermal melanocytosis
- Affects mostly persons of African, Hispanic, or Asian descent
- Five times more common in females
- Approximately 5%–13% of cases are bilateral
- Present at birth typically
- Rarely can present at puberty
- Can slowly enlarge
- Diffuse flat, irregularly shaped regions of blue/gray pigmentation of periocular skin (Figure 1)
- Patchy slate gray pigmentation on episclera and uvea in oculodermal melanocytosis
- Usually diagnosed based on clinical appearance
- Biopsy is performed on any suspicious lesion to confirm diagnosis and rule out malignancy.
Testing for staging or fundamental impairment
- Close follow-up required to monitor for uveal melanoma in oculodermal melanocytosis
- Melanoma more often in whites, but also reported in patients of African, Hispanic, or Asian descent
Figure 1. Clinical photo of a right-sided area of light flat pigmentation in the lateral upper and lower lids, lateral canthus, and temporal skin, consistent with nevus of Ota. Image courtesy Michael J. Hawes, MD.
African, Hispanic, or Asian descent
- Solar lentigo
- Lentigo simplex (brown-black small, less than 3 mm typically, flat macules)
- Blue nevus: dark, blue-gray, dome-shaped, elevated lesion that is congenital or arises during early childhood
- Represents a localized proliferation of dermal melanocytes
- Measures up to 10 mm in diameter
- Malignant transformation can occur rarely
- Malignant melanoma
- Lentigo maligna
- Dysplastic nevus (nevus of Clark)
- Spitz nevus
- Giant pigmented nevus (typically hairy)
Patient management: treatment and follow-up
Malignant transformation can occur, especially in white patients.
Topical hydroquinone 2%–4% or kojic acid preparations can offer mild improvement by bleaching the melanin pigment. Patient must understand that the lesions often recur with cessation of bleaching agents.
- Biopsy of any suspicious lesions
- Incisions placed in relaxed skin tension lines
- Reconstruction with flaps
Other management considerations
- Camouflage makeup
- Orange color correctors counterbalance blue pigmentation.
- Dermabrasion can remove epidermal and superficial dermal melanin, with mild to moderate improvement of appearance.
- Cryotherapy historically was used to treat nevus of Ota by suppressing the function of epidermal melanocytes as well as by direct cryonecrosis of dermal melanocytes.
- Laser treatment can selectively target cells containing pigment.
- Q-switched lasers, and intense pulsed light, broad band light (515‑nm filter) treatments are most popular and can be highly successful.
- Risks of hypopigmentation, incomplete treatment, recurrence
Common treatment responses, follow-up strategies
- Regular ophthalmology follow-up in patients with nevus of Ota to screen for uveal melanoma
- Seek medical attention if skin lesion changes in size or color
Preventing and managing treatment complications
- Incomplete improvement
- Recurrences common, particularly with topical bleaching agents
- Scarring, hypopigmentation, hyperpigmentation from laser treatments
- Repeat treatments can provide additional resolution
- Approximately 1 in 400 patients with oculodermal melanocytosis develop uveal malignant melanoma
- Cutaneous lesions rarely undergo malignant degeneration
- Mongolian spots affecting skin of sacrococcygeal region tend to persist in patients also affected with bilateral nevus of Ota
- First reported by Dr. M. T. Ota of Japan in 1939
- Intraocular uveal melanoma associated with oculodermal melanocytosis
- Laser treatment options for management, with subsequent results, risks, recurrence rates
References and additional resources
- AAO, Basic and Clinical Science Course. Section 7: Orbit, Eyelid and Lacrimal, 2013-2014.
- Anderson RR, Parrish JA. Selective photothermolysis: precise microsurgery by selective absorption of pulsed radiation. Science. 1983; 220:524-527.
- Chan HH, Leung RSC, Ying SY, Lai CF, Chua J, Kono T. Recurrence of nevus of Ota after successful treatment with Q-switched lasers. Arch Dermatol. 2000; 136:1175-1176.
- Chan HH, Ying SY, Ho WS, et al. An in vivo trial comparing the clinical efficacy and complications of Q-switched 755 nm alexandrite and Q-switched 1064 nm Nd:YAG lasers in the treatment of nevus of Ota. Dermatol Surg. 2000;26:919-922.
- Font RL, Reynolds AM, Jr, Zimmerman LE. Diffuse malignant melanoma of the iris in the nevus of Ota. Arch Ophthalmol. 1967; 77(4):513–518.
- Geronemus RG. Q-switched ruby laser therapy of nevus of Ota. Arch Dermatol. 1992; 128:1618-1622.
- Gonder JR, Ezell PC, Shields JA, et al. Ocular melanocytosis: a study to determine the prevalence rate of ocular melanocytosis. Ophthalmol. 1982; 89:950-952.
- Hosaka Y, Onizuka T, Ichinose M, et al. Treatment of naevus of Ota by liquid nitrogen cryotherapy. Plast Reconstr Surg. 1995; 95:703-711.
- Kono T, Nozaki M, Chan HH, et al. A retrospective study looking at the long-term complications of Q-switched ruby laser in the treatment of nevus of Ota. Lasers Surg Med. 2001; 29:156-159.
- McCormick SA, DeLuca, RL: Tumors of Melanocytic Origin. In: Mannis MJ, Macsai MS, Huntley AC, eds. Eye and Skin Disease, Philadelphia, Lippincott-Raven, 1996: 381-385.
- Patel BC, Egan CA, Lucius RW, et al. Cutaneous malignant melanoma and oculodermal melanocytosis (nevus of Ota): report of a case and review of the literature. J Am Acad Dermatol. 1998; 38:862-865.
- Stuart C. Naevus of Ota. Br J Dermatol. 1955; 67:317.
- Teekhasaenee C, Ritch R, Rutnin U, Leelawongs N. Ocular findings in oculodermal melanocytosis. Arch Ophthalmol. 1990;108:1114-1120.
- Yamamoto T. Malignant melanoma of the choroid in the nevus of Ota. Ophthalmologica. 1969; 159(1):1–10.