AUTHOR AND EDITOR INFORMATION

Section 1 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

INTRODUCTION

Section 2 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

Background

Nevus of Ota, which originally was described by Ota and Tanino in 1939, is a hamartoma of dermal melanocytes. Clinically, nevus of Ota presents as a blue or gray patch on the face, which is congenital or acquired and is within the distribution of the ophthalmic and maxillary branches of the trigeminal nerve. The nevus can be unilateral or bilateral, and, in addition to skin, it may involve ocular and oral mucosal surfaces.

Nevus of Ito, initially described by Minor Ito in 1954, is a dermal melanocytic condition affecting the shoulder area. Nevus of Ito often occurs in association with nevus of Ota in the same patient but is much less common, although the true incidence is unknown.

Pathophysiology

The etiology and pathogenesis of nevi of Ota and Ito are not known. Although unconfirmed, nevus of Ota and other dermal melanocytic disorders, such as nevus of Ito, blue nevus, and mongolian spots, may represent melanocytes that have not migrated completely from the neural crest to the epidermis during the embryonic stage. The variable prevalence among different populations suggests genetic influences, although familial cases of nevus of Ota are exceedingly rare. The 2 peak ages of onset in early infancy and in early adolescence suggest that hormones are a factor in the development of this condition. The observation of dermal melanocytes in close proximity with nerve bundles in nevus of Ito suggests that the nervous system is a factor in the development of nevus of Ito, although the true pathogenesis remains unknown.

Mortality/Morbidity

Nevus of Ota can cause facial disfigurement, resulting in emotional and psychologic distress. In rare cases, melanoma, which can be life threatening, has been reported to arise from nevus of Ota. Glaucoma also has been associated with nevus of Ota.

Nevus of Ito usually does not have symptoms and causes little cosmetic concern to the patients; however, sensory changes occasionally are present in the lesion.

Race

• Nevi of Ota and Ito occur most frequently in Asian populations, with an estimated prevalence of 0.2-0.6% for nevus of Ota in Japanese persons. Nevus of Ito is less common than nevus of Ota, although the true incidence is unknown.
• Other ethnic groups with increased prevalence include Africans, African Americans, and East Indians.
• Nevi of Ota and Ito are uncommon in whites.

Sex

• Male-to-female ratio is 1:4.8 for nevus of Ota. The ratio for nevus of Ito is unknown.

Age

• The first peak of onset of nevus of Ota occurs in infancy, with as many as 50% of nevus of Ota cases present at birth. The onset for nevus of Ito is at birth or shortly after.
• The second peak of onset for nevus of Ota is seen during adolescence.
• Isolated cases of delayed-onset nevi of Ota that first appear in adults, including in older patients, have been reported.

CLINICAL

Section 3 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

History

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
• • Nevus of Ito
  • Phakomatosis pigmentovascularis
  • Nevus flammeus
  • Sturge-Weber syndrome
  • Neurofibromatosis and leptomeningeal melanosis
• Malignant melanoma
• • More than 60 cases of malignant melanoma (56 in whites, 4 in Japanese) in association with nevus of Ota have been described in the literature as follows:
  • • Skin - 10 cases
    • Meninges - 12 cases
    • Ocular tissues - 40 cases
  • To date, only 1 case of malignant degeneration of nevus of Ito has been described and involved a 78-year-old white man.
• Ocular abnormalities (ocular acuity normal)
• • Pigmentation of the sclera, cornea, retina, and optic disc
  • Cavernous hemangiomas of the optic disc
  • Elevated intraocular pressure
  • Glaucoma (10.3%)
  • Ocular melanoma

Physical

Table. Clinical and Histologic Features for Differential Diagnoses of Nevi of Ota and Ito

Condition	Onset	Appearance	Location	Histology
Nevi of Ota and Ito	Birth or early adolescence	Blue or gray speckled coalescing macules or patches	For nevus of Ota, unilateral, rarely bilateral, on forehead, temple, zygomatic, or periorbital areas; for nevus of Ito, the shoulder and upper arm areas	Increased dermal melanocytes, with surrounding fibrosis and melanophages
Mongolian spot	Birth	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
Melasma	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
Phytophotodermatitis	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. 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%. 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, or Hori nevus. Some clinicians consider Hori nevus to be a distinct entity that is separate from nevus of Ota. 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.

Causes

The cause of nevi of Ota and Ito is unknown.

DIFFERENTIALS

Section 4 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

WORKUP

Section 5 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

Other Tests

• Consider ophthalmologic examination and follow-up care for nevus of Ota because of a reported 10% association of nevus of Ota with increased intraocular pressure.

Histologic Findings

Histologic findings for nevi of Ota and Ito are similar. Overlying epidermis is normal. In the papillary and upper reticular dermis, dendritic melanocytes are present and surrounded by fibrous sheaths (which are not present in other dermal melanocytosis, such as blue nevus or mongolian spots). Dermal melanophages may be present.

Nevi of Ota have been classified histologically into 5 types based on the locations of the dermal melanocytes, which are (1) superficial, (2) superficial dominant, (3) diffuse, (4) deep dominant, and (5) deep.

This histologic classification correlates clinically with the observation that the more superficial lesions tend to be located on the cheeks, while deeper lesions occur on periorbital areas, the temple, and forehead.

TREATMENT

Section 6 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

Medical Care

Cosmetic camouflage makeup can minimize the disfiguring facial pigmentation resulting from nevus of Ota. Otherwise, topical therapy is of no value in the medical treatment of nevi of Ota and Ito.

Surgical Care

• Laser surgery
  • Pulsed Q-switched laser surgery is unquestionably the current treatment of choice for nevi of Ota and Ito, and it works via selective photothermal and photomechanical destruction of dermal melanocytes and melanophages.
  • High success rates and minimal side effects have been reported with the Q-switched ruby, Q-switched alexandrite, and Q-switched Nd:YAG lasers.
  • After 4-8 treatments, skin pigmentation is reduced dramatically or removed in 90-100% of cases, with a less than 1% risk of scarring.
• Other surgical methods (currently have been superseded by laser surgery)
• • Cryotherapy
  • Microsurgery
  • Dermabrasion (alone or combined with other modalities, such as carbon dioxide snow, autologous epithelial grafting)
  • Sequential dry ice epidermal peeling

Consultations

Ophthalmologist - For nevus of Ota, which may be associated with a higher incidence of ocular disease

FOLLOW-UP

Section 7 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

Further Outpatient Care

• No special dermatology outpatient follow-up care is required; however, an ophthalmologist should examine patients with nevus of Ota periodically for the development of glaucoma.

Complications

• 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, or mucosal tissues.
• Ophthalmologic follow-up care is necessary for patients with increased intraocular pressure.

Prognosis

• Without treatment, the skin lesions are permanent.
• The development of glaucoma in patients with nevus of Ota can be as high as 10%.
• Malignant degeneration has been described rarely in white patients with both nevus of Ota and nevus of Ito.

Patient Education

• Make patients aware of the risk associated with the development of glaucoma. Instruct patients to schedule periodic follow-up visits with an ophthalmologist.
• Instruct patients to report any unusual symptoms or changes to the lesional areas to a physician, since a small risk for malignant degeneration exists.

MISCELLANEOUS

Section 8 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

Medical/Legal Pitfalls

• Failure to recognize the potential for malignant degradation of nevi of Ota and Ito, especially in white patients, may result in medicolegal complications.

REFERENCES

Section 9 of 9

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

• Anderson RR. Lasers in dermatology--a critical update. J Dermatol. Nov 2000;27(11):700-5. [Medline].
• Chan HH, Leung RS, Ying SY, et al. A retrospective analysis of complications in the treatment of nevus of Ota with the Q-switched alexandrite and Q-switched Nd:YAG lasers. Dermatol Surg. Nov 2000;26(11):1000-6. [Medline].
• Hidano A, Kajima H, Ikeda S, et al. Natural history of nevus of Ota. Arch Dermatol. Feb 1967;95(2):187-95. [Medline].
• Hirayama T, Suzuki T. A new classification of Ota''s nevus based on histopathological features. Dermatologica. 1991;183(3):169-72. [Medline].
• Hosaka Y, Onizuka T, Ichinose M, et al. Treatment of nevus Ota by liquid nitrogen cryotherapy. Plast Reconstr Surg. Apr 1995;95(4):703-11. [Medline].
• Ito M. Studies on melanin XXII. Nevus fuscocaeruleus acromio-deltoideus. Tohoko J Exper Med. 1954;60:10.
• 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. May 1998;38(5 Pt 2):862-5. [Medline].
• Teekhasaenee C, Ritch R, Rutnin U, Leelawongs N. Glaucoma in oculodermal melanocytosis. Ophthalmology. May 1990;97(5):562-70. [Medline].
• van Krieken JH, Boom BW, Scheffer E. Malignant transformation in a naevus of Ito. A case report. Histopathology. Jan 1988;12(1):100-2. [Medline].
• Watanabe S, Takahashi H. Treatment of nevus of Ota with the Q-switched ruby laser. N Engl J Med. Dec 29 1994;331(26):1745-50. [Medline].

Article Last Updated: Jan 12, 2007