Establishing the diagnosis

Etiology

• Arises from a benign localized proliferation of dendritic dermal melanocytes typically located in the mid-dermis

Epidemiology

• Not inherited condition
• Prevalence is difficult to determine.

History

• Congenital or develops during childhood

Clinical features

• Dark blue-gray to blue-black lesions, typically elevated
• Dome-shaped
• Located beneath the epidermis
• Because of deeper location, has a blue to gray coloration due to scatter of light reflected by the deep pigmentation (Tyndall effect)
• Usually less than 10 mm in diameter
• Distribution is head, neck, face, feet, and buttocks.
• About 20% of cases classified as cellular blue nevi and are larger and more dome shaped.

Testing

• Usually diagnosed based on clinical appearance
• Biopsy is performed on any suspicious lesion to rule out malignancy.

Risk factors

• None

Differential diagnosis

• Nevus
• Lentigo simplex
• Solar lentigo
• Malignant melanoma

Patient management: treatment and follow-up

Natural history

• Malignant potential extremely low

Medical therapy

• Usually not medically treated

Surgery

• Excision of lesion
• Laser treatment

Preventing and managing treatment complications

• Scarring or recurrence after excision
• Take care to excise entire lesion, meticulous wound closure.

Patient instructions

• If lesion enlarges or changes, see physician for evaluation and excisional biopsy.

References and additional resources

1. 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.
2. Milgraum SS, Cohen ME, Auletta MJ: Treatment of blue nevi with the Q-switched ruby laser. J Am Acad Dermatol. 1995; 32:307-310,

Establishing the diagnosis

Etiology

• 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

Epidemiology

• Congenital
• Affects mostly persons of African, Hispanic, or Asian descent
• Five times more common in females
• Approximately 5%–13% of cases are bilateral

History

• Present at birth typically
• Rarely can present at puberty
• Can slowly enlarge

Clinical features

• 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

Testing

• 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.

Risk factors

African, Hispanic, or Asian descent

Differential diagnosis

• Nevus
• 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

Natural history

Malignant transformation can occur, especially in white patients.

Medical therapy

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.

Surgery

• 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.
• Risks of tissue scarring
• 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

Disease-related complications

• 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

Historical perspective

• 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

1. AAO, Basic and Clinical Science Course. Section 7: Orbit, Eyelid and Lacrimal, 2013-2014.
2. Anderson RR, Parrish JA. Selective photothermolysis: precise microsurgery by selective absorption of pulsed radiation. Science. 1983; 220:524-527.
3. 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.
4. 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.
5. 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.
6. Geronemus RG. Q-switched ruby laser therapy of nevus of Ota. Arch Dermatol. 1992; 128:1618-1622.
7. 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.
8. Hosaka Y, Onizuka T, Ichinose M, et al. Treatment of naevus of Ota by liquid nitrogen cryotherapy. Plast Reconstr Surg. 1995; 95:703-711.
9. 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.
10. 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.
11. 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.
12. Stuart C. Naevus of Ota. Br J Dermatol. 1955; 67:317.
13. Teekhasaenee C, Ritch R, Rutnin U, Leelawongs N. Ocular findings in oculodermal melanocytosis. Arch Ophthalmol. 1990;108:1114-1120.
14. Yamamoto T. Malignant melanoma of the choroid in the nevus of Ota. Ophthalmologica. 1969; 159(1):1–10.

Establishing the diagnosis

Etiology

• Dermal melanocytes proliferate in the region of the ophthalmic (V₁) and maxillary (V₂) dermatomes of the trigeminal nerve.
• When patchy slate gray pigmentation also occurs on the episclera and uvea, as occurs in 2/3rds of affected patients, the disease is known as oculodermal melanocytosis (nevus of Ota).

Epidemiology

• Congenital
• Affects mostly persons of African, Hispanic, or Asian descent
• More common in females
• Approximately 5%–13% of cases are bilateral.

History

• Present at birth

Clinical features

• Diffuse, flat, irregularly shaped regions of blue/gray pigmentation of periocular skin
• Patchy slate gray pigmentation on episclera and uvea in oculodermal melanocytosis

Testing

• Usually diagnosed based on clinical appearance
• Biopsy is performed on any suspicious lesion to confirm diagnosis and rule out malignancy.

Risk factors

• African, Hispanic, or Asian descent

Differential diagnosis

• Nevus
• Solar lentigo
• Lentigo simplex
• Blue nevus
• Malignant melanoma

Patient management: treatment and follow-up

Natural history

• Malignant transformation can occur (uveal melanoma more common than cutaneous), especially in white patients.
• Increased risk for glaucoma in effected eye

Medical therapy

• Routine follow-up to screen for glaucoma and malignant transformation

Surgery

• Biopsy of any suspicious lesions

Laser therapy

• Several reports of laser treatment reducing the appearance of nevus of Ota

Disease-related complications

• Approximately 1 in 400 patients with oculodermal melanocytosis develop a uveal malignant melanoma.
• Cutaneous lesions rarely undergo malignant degeneration.
• Unilateral glaucoma
• Mongolian spots affecting skin of sacrococcygeal region tend to persist in patients also affected with bilateral nevus of Ota.

Patient instructions

• Regular ophthalmology follow-up in patients with nevus of Ota to screen for glaucoma and uveal melanoma
• Seek medical attention if skin lesion changes in size or color.

References and additional resources

1. AAO, Basic and Clinical Science Course. Section 7: Orbit, Eyelid and Lacrimal, 2010-2011.
2. 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.
3. Seo HM, Choi CW, and Kim WS. Beneficial effects of early treatment of nevus of Ota with low-fluence 1,064-nm Q-switched Nd:YAG laser. Dermatol Surg, 2015; 41:142-148.
4. Choi JE, Lee JB, Park KB, et al. A retrospective analysis of the clinical efficacies of Q-switched Alexandrite and Q-switched Nd:YAG lasers in the treatment of nevus of Ota in Korean patients. J Dermatolog Treat, 9: 1-6, 2014.

Establishing the diagnosis

Etiology

• Derived from epidermal melanocytes that extrude more than the usual amount of pigment into the epidermal basal layer and produce hyperpigmentation of basal layer of epidermis
• Not true neoplasm

Epidemiology

• Perhaps most common of all skin lesions
• More common in fair-skinned individuals, though more pigmented individuals can be affected as well.
• Lesions darken with sunlight exposure.
• Males and females affected equally

History

• Usually present since childhood
• Do not grow in size
• Darken with sun exposure
• Nonpainful

Clinical features

• Benign small flat tan to brown lesion of skin
• Small (1–3 mm in diameter), flat, tan to brown macules on skin
• Usually appear on malar areas
• Can present on eyelids or conjunctiva
• Darken with sun exposure

Testing

• Usually diagnosed based on clinical appearance
• Biopsy is performed on any suspicious lesion to confirm diagnosis and rule out malignancy.

Risk factors

• Fair-skinned individuals
• Sun exposure

Differential diagnosis

• Nevi
• Lentigo simplex
• Malignant melanoma
• Seborrheic keratosis
• Solar lentigo

Patient management: treatment and follow-up

Natural history

• Benign lesions, do not grow or transform into malignant lesions
• Prognosis is excellent

Medical therapy

• Observation
• Avoidance of UV exposure: hat, sunglasses, sunscreen
• Topical hydroquinone, tretinoin, or other chemicals that interact with melanin synthesis pathway

Surgery

• Biopsy any suspicious lesions (growing, bleeding, ulceration).
• Most do not require surgery as they are multiple and diffuse.

Preventing and managing treatment complications

• Scarring can occur after excision.
• Tretinoin creams can cause redness, irritation and photosensitivity; increase dose slowly.

Patient instructions

• UV exposure avoidance to prevent darkening of lesions

1. AAO, Basic and Clinical Science Course. 2010–2011.
2. 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.