Nevi of Ota and Ito Clinical Presentation

Updated: Mar 26, 2019
  • Author: Omobola Onikoyi, DO, MSc; Chief Editor: Dirk M Elston, MD  more...
  • Print


After onset, nevus of Ota may slowly and progressively enlarge and darken in color, and its appearance usually remains stable once adulthood is reached. The color or perception of the color of nevus of Ota may fluctuate according to personal and environmental conditions, such as fatigue, menstruation, insomnia, and cloudy, cold, or hot weather conditions. Nevus of Ota can be associated with other cutaneous disorders and ocular disease. Nevus of Ito can be associated with sensory changes in the involved skin.

Benign cutaneous and leptomeningeal conditions associated with nevus of Ota are as follows [21] :

  • Nevus of Ito

  • Phakomatosis pigmentovascularis

  • Nevus flammeus

  • Sturge-Weber syndrome

  • Klippel-Trenaunay syndrome

  • Takayasu disease

  • Neurofibromatosis and leptomeningeal melanosis

Malignant degeneration of nevus of Ota is rare. More than 100 cases of malignant melanoma in association with nevus of Ota have been described in the literature as follows [22] :

To date, 13 cases of malignant degeneration of nevus of Ito have been described. [19]

Ocular abnormalities (ocular acuity normal) are as follows:

  • Pigmentation of the sclera, cornea, iris, retina, and optic disc [33]

  • Cavernous hemangiomas of the optic disc

  • Elevated intraocular pressure

  • Glaucoma (10.3%) [34]

  • Ocular melanoma


Physical Examination

Table. Clinical and Histologic Features for Differential Diagnoses of Nevi of Ota and Ito (Open Table in a new window)






Nevi of Ota and Ito

Birth or early adolescence

Blue or gray speckled coalescing macules or patches

Nevus of Ota: Unilateral, rarely bilateral, on forehead, temple, zygomatic, or periorbital areas

Nevus of Ito: Shoulder and upper arm areas

Increased dermal melanocytes, with surrounding fibrosis and melanophages

Mongolian spot


Poorly demarcated large blue-to-gray patches that tend to spontaneously resolve by age 3-6 y

Most frequently on lumbosacral areas, buttocks, and rarely, other areas

Increased dermal melanocytes; no surrounding fibrosis

Blue nevus

Congenital or acquired

Blue papules or plaques

Anywhere on skin

Dermal nodular proliferation of heavily pigmented spindle cells

Acquired nevus of Ota-like macules (Hori nevus)

Acquired, presenting in adulthood

Gray macules or patches

Usually bilateral and symmetrical; over the cheeks, temples, root of the nose, alae nasi, eyelids, and forehead

Diffuse upper-dermal melanocytosis


Acquired; may be associated with pregnancy and other estrogen excess stages

Well-to-poorly demarcated and irregularly outlined brown-to-gray brown patches

Maxillary and zygomatic areas on face

No increase in dermal melanocytes; presence of melanophages

Lentigo maligna

Acquired; presenting usually after fifth decade of life

Brown patches, usually with pigmentary variegation

Photodistribution, particularly within zygomaticomaxillary areas

Atypical melanocytes in nests at dermal-epidermal junction, with pagetoid spread

Actinic lentigo

Acquired; usually after fifth decade of life

Well-demarcated brown papules or plaques

Photodistribution, especially on face

Elongation of rete ridges; basal layer hyperpigmentation; slight increase of melanocyte number along basal layer


Acquired; exposure to certain plants or cosmetics

Gray-to-brown macules and patches

Photodistribution, according to sites of contact with photosensitizer

Dermal melanophages

Drug-induced hyperpigmentation

Acquired; following drug exposure (eg, minocycline, amiodarone, gold)

Variable according to offending drugs

Variable according to specific offending drugs

Variable but may involve presence of dermal melanophages; pigmentation of basal keratinocytes

Exogenous ochronosis (rare)

Adulthood; following topical application of hydroquinone

Irregularly shaped blue-to-gray patches or macules

Areas corresponding to exposure to hydroquinone

Yellow banana-shaped spindle cells in papillary dermis

Ochronosis (alkaptonuria, rare)

First decade of life

Blue-gray discoloration of ear cartilage, tip of nose, and sclera

Symmetrical distribution over cartilage, nose, cheeks, and extensor tendons of hands, as well as flexural areas

Yellow-to-brown pigmentary granules within dermal macrophages

Nevus of Ota most frequently presents as blue-to-gray speckled or mottled coalescing macules or patches affecting the forehead, temple, malar area, or periorbital skin. [35] Nevus of Ito presents as a patch on the shoulder or upper arms with blue, gray, or brown pigmentation. Most cases of nevus of Ota are unilateral (90%), although pigmentation is present bilaterally in 5-10%. [36] Nevus of Ito usually is unilateral. In addition to skin, pigmentation of nevus of Ota may involve oral mucosa and ocular structures such as the sclera, retrobulbar fat, cornea, and retina.

Clinically, nevus of Ito is similar to nevus of Ota, except that it typically presents over the shoulder girdle region.

Specific variants of nevus of Ota have been described in the literature under the names of nevus fuscoceruleus zygomaticus, plaque-type variant of blue nevus. Differential features of these conditions are related to the following:

  • Location of patch or macules

  • Extent of involvement

  • Age of onset

  • Tendency to occur as familial cases

  • Presence of a papular component

Pathology and response to therapy appear similar for all forms of nevus of Ota. The pathology of nevus of Ito is similar to that of nevus of Ota.

Tanino created the following four classifications of nevus of Ota based on the extent of disease and localization of distribution:

  • Type I (mild): Type A has an orbital distribution, is periocular, and is localized to the upper/lower eyelid or temporal region. Type B is localized to the zygomatic region. Type C is localized only to the forehead. Type D is localized only to the nasal ala.
  • Type II (moderate): Lesions may be distributed as those seen in type I, but to a more extensive degree.
  • Type III (intensive): Lesions have forehead, eyebrow, nose, and scalp involvement.
  • Type IV: Lesions have a bilateral distribution.


Studies have shown that patients with dermal melanocytosis subsequently went on to develop uveal melanoma had an increased risk for metastasis double that of patients without melanocytosis. [37]

Skin biopsies are warranted if clinical changes are suspected of malignant transformation (eg, ulceration, new papular lesions, variegations in color) within the involved skin, ocular tissues, or mucosal tissues.

Ophthalmologic follow-up care is necessary for patients with increased intraocular pressure. Biannual follow up is recommended.