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Dermatology > BENIGN NEOPLASMS
Nevi of Ota and Ito
Article Last Updated: Jul 14, 2008
AUTHOR AND EDITOR INFORMATION
Section 1 of 9  
Author: Harvey Lui, MD, FRCPC, Professor and Head, Department of Dermatology and Skin Science, Vancouver General Hospital, University of British Columbia; Medical Director, The Skin Centre, Lions Laser Skin Centre and Psoriasis and Phototherapy Clinic, Vancouver General Hospital
Harvey Lui is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, American Society for Laser Medicine and Surgery, American Society for Photobiology, Canadian Dermatology Association, Canadian Dermatology Foundation, Canadian Medical Association, College of Physicians and Surgeons of British Columbia, Dermatology Foundation, European Academy of Dermatology and Venereology, National Psoriasis Foundation, North American Hair Research Society, and Photomedicine Society
Coauthor(s):
Youwen Zhou, MD, PhD, FRCP(C), Associate Professor, Department of Dermatology and Skin Science, University of British Columbia, Vancouver, Canada
Editors: Sungnack Lee, MD, Vice President of Medical Affairs, Professor, Department of Dermatology, Ajou University School of Medicine, Korea; Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center; Christen M Mowad, MD, Associate Professor, Department of Dermatology, Geisinger Medical Center; Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania; William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System
Author and Editor Disclosure
Synonyms and related keywords:
hamartoma, nevus of Ota, nevus of Ito, Hori nevus, Hori's nevus, nevus fuscoceruleus zygomaticus, plaque-type variant of blue nevus, nevus fuscoceruleus acromiodeltoideus
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 Ito1 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.
Additionally, the eMedicine article Nevi, Melanocytic may be of interest.
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.
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 follows2:
- 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.3
- 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%)4
- Ocular melanoma
Physical
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.
Blue Nevi
Lentigo
Malignant Melanoma
Melasma
Mongolian Spot
Ochronosis
Phytophotodermatitis
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.5 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.
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 surgery6
- 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 adverse effects have been reported with the Q-switched ruby,7 Q-switched alexandrite,8, 9 and Q-switched Nd:YAG lasers.8
- 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)
- Cryotherapy10
- Microsurgery
- Dermabrasion (alone or combined with other modalities, such as carbon dioxide snow, autologous epithelial grafting)
- Sequential dry ice epidermal peeling
- The Medscape Dermatologic Surgery Resource Center may be helpful.
Consultations
Ophthalmologist - For nevus of Ota, which may be associated with a higher incidence of ocular disease
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.
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.
- Ito M. Studies on melanin XXII. Nevus fuscocaeruleus acromio-deltoideus. Tohoko J Exper Med. 1954;60:10.
- Patel BC, Egan CA, Lucius RW, Gerwels JW, Mamalis N, Anderson RL. 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].
- 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].
- Teekhasaenee C, Ritch R, Rutnin U, Leelawongs N. Glaucoma in oculodermal melanocytosis. Ophthalmology. May 1990;97(5):562-70. [Medline].
- Hirayama T, Suzuki T. A new classification of Ota's nevus based on histopathological features. Dermatologica. 1991;183(3):169-72. [Medline].
- Anderson RR. Lasers in dermatology--a critical update. J Dermatol. Nov 2000;27(11):700-5. [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].
- Chan HH, Leung RS, Ying SY, Lai CF, Kono T, Chua JK, 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].
- Wang HW, Liu YH, Zhang GK, Jin HZ, Zuo YG, Jiang GT, et al. Analysis of 602 Chinese cases of nevus of Ota and the treatment results treated by Q-switched alexandrite laser. Dermatol Surg. Apr 2007;33(4):455-60. [Medline].
- Hosaka Y, Onizuka T, Ichinose M, Yoshimoto S, Okubo F, Hori S, et al. Treatment of nevus Ota by liquid nitrogen cryotherapy. Plast Reconstr Surg. Apr 1995;95(4):703-11. [Medline].
- Hidano A, Kajima H, Ikeda S, Mizutani H, Miyasato H, Niimura M. Natural history of nevus of Ota. Arch Dermatol. Feb 1967;95(2):187-95. [Medline].
Nevi of Ota and Ito excerpt Article Last Updated: Jul 14, 2008
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