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Background

These are relatively uncommon dark discolorations occurring around the eye and shoulders. The course is usually benign but can be disfiguring and a cosmetic problem. Newer modalities such as lasers have produced excellent cosmetic results.

OUTLINE

Epidemiology  
Disease Associations  
Pathogenesis  
Laboratory/Radiologic/Other Diagnostic Testing  
Gross Appearance and Clinical Variants  
Histopathological Features and Variants  
Special Stains/
Immunohistochemistry/
Electron Microscopy
 
Differential Diagnosis  
Prognosis  
Treatment  
Commonly Used Terms  
Internet Links  

 

EPIDEMIOLOGY CHARACTERIZATION
SYNONYMS  
INCIDENCE/
PREVALENCE
 
AGE  
Late-onset Ito's nevus: An uncommon acquired dermal melanocytosis.

Department of Dermatology, Fundación Jiménez Díaz, Universidad Autónoma, Madrid, Spain.

 

J Cutan Pathol. 2007 Aug;34(8):640-3. Abstract quote

Dermal melanocytoses comprise a variety of congenital and acquired conditions characterized by a sparse population of intradermal dendritic, variably pigmented, spindle-shaped melanocytes. While Mongolian spot, Ota's and Ito's nevi are usually present at birth or appear around puberty; acquired dermal melanocytoses that appear in adult life are extremely rare. They include the facial lesions of acquired bilateral nevus of Ota-like macules, also named Hori's nevus, and the acquired unilateral nevus of Ota, also known as Sun's nevus. Uncommon extrafacial examples of acquired dermal melanocytoses include lesions involving upper extremities, wrist, back, lower extremities and dorsal aspects of the hands and feet. They are more prevalent among Asian women. In general, dermal melanocytoses are rare lesions in Caucasian patients and acquired variants are exceedingly uncommon.

We report a rare example of acquired Ito's nevus that appeared in a Caucasian elderly woman and review the literature about acquired dermal melanocytoses.
SEX  
GEOGRAPHY  
EPIDEMIOLOGIC ASSOCIATIONS  
Bilateral naevus of Ota: a rare manifestation in a Caucasian.

Turnbull JR, Assaf Ch, Zouboulis C, Tebbe B.

Department of Dermatology, University Medical Center Benjamin Franklin, The Free University of Berlin, Fabeckstrasse 60-62, D-12200 Berlin, Germany.
J Eur Acad Dermatol Venereol. 2004 May;18(3):353-5. Abstract quote  

The naevus of Ota (naevus fusculocoeruleus ophthalmomaxillaris) was first described by the Japanese dermatologist M. T. Ota in 1939. It has a reported incidence of 0.2% to 1% in the Japanese population. It usually occurs in the skin innervated by the first or second branch of the trigeminal nerve. The naevus comprises dermal melanocytes and is congenital or acquired during adolescence. Commonly associated lesions include scleral melanocytosis and other ocular manifestations as well as lesions of the tympanic membrane, oral and intranasal mucosa and leptomeninges.

Diseases associated with Ota's naevus in rare cases are open-angle glaucomas and melanoma. The naevus of Ota in Europeans is a rare manifestation.

We report the very rare case of a bilateral naevus of Ota associated with enoral melanocytosis in a white European person.

 

DISEASE ASSOCIATIONS CHARACTERIZATION
DEAFNESS  
Nevus of Ota associated with ipsilateral deafness.

Alvarez-Cuesta CC, Raya-Aguado C, Vazquez-Lopez F, Garcia PB, Perez-Oliva N.

Department of Dermatology, Hospital Central de Asturias, Oviedo, Spain.
J Am Acad Dermatol. 2002 Nov;47(5 Suppl):S257-9. Abstract quote  

Nevus of Ota is a benign pigmentary disorder that involves the skin innervated by the first and second branches of the trigeminal nerve. It is a dermal melanocytosis frequent in Oriental persons but uncommon in white persons.

We report a case of nevus of Ota in a white woman emphasizing the wide extension of the pigmentation and its association with ipsilateral sensorineural hypoacusia.
MELANOCYTOMA  
Meningeal melanocytoma associated with ipsilateral nevus of Ota presenting as intracerebral hemorrhage: case report.

Hino K, Nagane M, Fujioka Y, Shiokawa Y.

Department of Neurosurgery, Kyorin University School of Medicine, Tokyo, Japan.
Neurosurgery. 2005 Jun;56(6):E1376; discussion E1376. Abstract quote  

OBJECTIVE AND IMPORTANCE: The authors report a rare case of meningeal melanocytoma presenting with unconsciousness, which was caused by an intracerebral hematoma and associated with a history of ipsilateral nevus of Ota.

CLINICAL PRESENTATION: A 75-year-old woman developed nevus of Ota in the first and second divisions of the right trigeminal nerve territory, which had been treated with a skin graft 40 years earlier. She noticed right exophthalmos but left it untreated for 2 years and then became comatose owing to orbital and intracranial tumors, the latter manifesting with hemorrhage.

INTERVENTION: She underwent craniotomy, during which the tumor was partially removed with intracerebral hematoma. Histopathologically, the tumor was diagnosed as meningeal melanocytoma. Western blot analysis demonstrated a retained protein expression of cell cycle inhibitor p16(INK4A) and a high level of antiapoptotic Bcl-2 in the resected tumor.

CONCLUSION: The combination of nevus of Ota and meningeal melanocytoma has been reported in only four cases in the literature, including the current case. This is the first case coinciding with intracerebral hemorrhage, suggesting the necessity for careful follow-up with radiological images.

 

PATHOGENESIS CHARACTERIZATION

 

LABORATORY/
RADIOLOGIC/
OTHER TESTS

CHARACTERIZATION
RADIOLOGIC  
LABORATORY MARKERS  

 

GROSS APPEARANCE/
CLINICAL VARIANTS
CHARACTERIZATION
GENERAL  
VARIANTS  
AGMINATED LENTIGINES  
Bilateral type of nevus of Ota presenting as agminated lentigines.

Kang HY, Kang WH.

Department of Dermatology, Ajou University School of Medicine, 5 Wonchon-dong, Paldal-ku, Suwon 442-721, Korea.

Eur J Dermatol. 2003 Mar-Apr;13(2):205-6. Abstract quote  

We report a 30-year-old female patient with a history of hyperpigmented macules of the face since the age of 7 years. The clinical appearance, brown macules in multiple segmental or grouped patterns, was suggested to be agminated lentigines. However, histologic examination of the pigmented macule revealed scattered melanocytes within the dermis.

We diagnosed this lesion as a bilateral type of nevus of Ota presenting as agminated lentigines. The differential diagnosis from acquired bilateral nevus of Ota-like macules was made.
BILATERAL  
Bilateral Ota naevus.

Ruiz-Villaverde R, Blasco Melguizo J, Buendia Eisman A, Serrano Ortega S.

Servicio de Dermatologia, Hospital Clinico San Cecilio, Av. Madrid s/n, 18012-Granada, Spain.
J Eur Acad Dermatol Venereol. 2003 Jul;17(4):437-9. Abstract quote  

We present the case of a 22-year-old woman, who had presented since the age of 15 a pale-blue spot spread on the right-hand side of her forehead and in her bulbar conjunctiva (first and second branches of the trigeminus nerve), consistent with Ota naevus. A few years later another with similar characteristics appeared on the other side of her forehead, cheek and sclera. No deafness, neurological defect nor visual loss were detected.

We comment on the rarity of this case because the patient is Caucasian and also we explain the main complications derived of this disease and consider the therapeutic options.
HORI'S NEVUS (ACQUIRED BILATERAL NEVUS OF OTA-LIKE MACULES)  
Acquired, bilateral nevus of Ota-like macules (ABNOM) associated with Ota's nevus: case report.

Park JH, Lee MH.

Department of Dermatology, College of Medicine, Kyung Hee University, Seoul, Korea.

J Korean Med Sci. 2004 Aug;19(4):616-8. Abstract quote  

Ota's nevus is mongolian spot-like macular blue-black or gray-brown patchy pigmentation that most commonly occurs in areas innervated by the first and second division of the trigeminal nerve.

Acquired, bilateral nevus of Ota-like macules (ABNOM) is located bilaterally on the face, appears later in life, is blue-brown or slate-gray in color. It is not accompanied by macules on the ocular and mucosal membranes. There is also debate as to whether ABNOM is part of the Ota's nevus spectrum.

We report an interesting case of ABNOM associated with Ota's nevus. A 36-yr-old Korean women visited our clinic with dark bluish patch on the right cheek and right conjunctiva since birth. She also had mottled brownish macules on both forehead and both lower eyelids that have developed 3 yr ago. Skin biopsy specimens taken from the right cheek and left forehead all showed scattered, bipolar or irregular melanocytes in the dermis. We diagnosed lesion on the right cheek area as Ota's nevus and those on both forehead and both lower eyelids as ABNOM by clinical and histologic findings.

This case may support the view that ABNOM is a separate entity from bilateral Ota's nevus.


Treatment of acquired bilateral nevus of Ota-like macules (Hori's nevus) using a combination of scanned carbon dioxide laser followed by Q-switched ruby laser.

Manuskiatti W, Sivayathorn A, Leelaudomlipi P, Fitzpatrick RE.

Department of Dermatology, Siriraj Hospital, Mahidol University, and Dermatology Associates/Cosmetic Laser Associates of San Diego County Inc.

 

J Am Acad Dermatol 2003 Apr;48(4):584-91 Abstract quote

BACKGROUND: Acquired bilateral nevus of Ota-like macules (Hori's nevus) is a dermal pigmented lesion commonly seen in middle-aged women of Asian descent. The Q-switched ruby laser (QSRL) has been used successfully to treat a variety of benign pigmented lesions. Multiple, sequential treatments are typically required for complete clearance of the dermal pigmented dermatoses.

OBJECTIVE: The purpose of this study was to determine the efficacy of QSRL in the treatment of Hori's nevus and the beneficial effect of epidermal ablation using the scanned carbon dioxide (CO(2)) laser before QSRL.

METHODS: A total of 13 women from Thailand with Hori's nevus were randomly treated with the scanned CO(2) laser followed by QSRL on one side of their face, and QSRL alone on the other side. The same fluence of QSRL was used on both sides in individual patients. The treatment response was objectively evaluated by measuring the melanin index using a Mexameter (Courage & Khazaka Electronic GmbH, Koln, Germany), and subjectively assessed by the patients before treatment and 3 and 16 months after treatment. Adverse sequelae of the treatment and the patients' tolerance were also evaluated at the same follow-up visit.

RESULTS: The 3- and 16-month posttreatment melanin index was significantly decreased compared with that of pretreatment on both treated sites and this corresponded to the patients' subjective evaluations. The response rate, defined as "the percentage of reduction in melanin index," was significantly higher on the sides treated with scanned CO(2) laser followed by QSRL, compared with the sides irradiated with QSRL alone at both follow-up visits. At the 3-month follow-up, the most common adverse effect was hypopigmentation, found in 15% (2 of 13) of the patients on the sites treated with QSRL alone, and on the sites treated with scanned CO(2) laser followed by QSRL (8%, 1/13). Erythema was observed in 15% (2/13) of the patients only on the sites that received combination treatment. However, no adverse sequelae were observed at the 16-month posttreatment follow-up.

CONCLUSION: Epidermal ablation with scanned CO(2) laser before the use of the pigment-specific laser may be an effective technique for increasing therapeutic efficacy in the treatment of dermal pigmented dermatoses.

PALATAL PIGMENTATION  
Oculodermal melanocytosis--Nevus of Ota (with palatal pigmentation).

Kannan SK.

Department of Oral Medicine & Radiology, Sree Mookambika Institute of Dental Sciences, Kulasekharam, Kanyakumari District, Tamilnadu--629161.
Indian J Dent Res. 2003 Oct-Dec;14(4):230-3. Abstract quote  

Nevus of Ota is a benign dermal melanocytosis, that involves facial skin as a macular discoloration, appearing as blotchy blue to blue gray.

It involves the upper portion of the face, especially over the periorbital area. It also involves ocular structures such as episclera, sclera, conjunctiva, cornea, retina, uveal tract and also nasopharynx, auricular mucosa, tymphanic membrane and dura. Glaucoma and malignant melanoma are the two known ocular complications in patients with Nevus of Ota. 12-18% of these patients exhibits palatal pigmentation.

Here we are reporting two cases of Oculodermal melanocytosis, one of which exhibited palatal pigmentation.

 

HISTOLOGICAL TYPES CHARACTERIZATION
GENERAL  
Dermal dendritic melanocytic proliferations: an update.

Zembowicz A, Mihm MC.

Dermatopathology Unit, Department of Pathology, Massachusetts General Hospital and Massachusetts Eye and Ear Infirmary, Harvard Medical School, Harvard, MA 02114, USA.
Histopathology. 2004 Nov;45(5):433-51. Abstract quote  

Dermal dendritic melanocytic proliferations are a broad group of congenital and acquired melanocytic lesions characterized by the presence of dermal spindled and dendritic cells resembling melanocytes migrating from the neural crest to the epidermis.

Historically, they were subdivided into dermal melanocytoses (naevus of Ota, Ito, Mongolian spot and related conditions), blue naevi and malignant blue naevi.

The purpose of this review is to provide an update on recent developments in the field with emphasis on new entities and their differential diagnosis.

 

SPECIAL STAINS/
IMMUNOPEROXIDASE/
OTHER
CHARACTERIZATION
SPECIAL STAINS  
IMMUNOPEROXIDASE  
ELECTRON MICROSCOPY  

 

DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES
BLUE NEVUS  
MONGOLIAN SPOT  
TATTOO  

 

PROGNOSIS CHARACTERIZATION
GLAUCOMA  
MALIGNANT MELANOMA  

 

TREATMENT CHARACTERIZATION
GENERAL  
LASER  
Treatment of 522 patients with Nevus of Ota with Q-switched Alexandrite laser.

Lu Z, Fang L, Jiao S, Huang W, Chen J, Wang X.

Department of Dermatology, Huashan Hospital, Medical College of Fudan University, Shanghai 200040, China.
Chin Med J (Engl). 2003 Feb;116(2):226-30. Abstract quote  

OBJECTIVE: To evaluate the clinical response of Nevus of Ota to Q-switched Alexandrite laser, and analyze factors that influence the treatment outcome.

METHODS: A total of 522 patients treated with Q-switched Alexandrite laser were included in the study. Single and multiple variate analyses of various factors were performed.

RESULTS: Satisfactory result was observed in all patients, none of whom developed scarring. Clinical response was improved with additional treatment sessions. The clinical response of the 20 - 27-week treatment interval group was significantly better than that of the 12 - 19-week interval group, but showed no significant difference as compared with both the 28 - 35 and > or = 36-week interval group. Zygomatic, buccal and frontal areas showed better response than ocular and temporal areas. Treatment session, interval, and fluence were significant factors identified by multivariate analysis.

CONCLUSIONS: Q-switched Alexandrite laser is an ideal method for treating Nevus of Ota without injury. The number of treatment sessions is more important than interval or fluence.
Nevus of Ota: clinical aspects and management.

Chan HH, Kono T.

The Division of Dermatology, Department of Medicine, University of Hong Kong, Room 802 Administration Building, Queen Mary Hospital, Hong Kong, PR China.
Skinmed. 2003 Mar-Apr;2(2):89-96; quiz 97-8. Abstract quote  

Nevus of Ota, or nevus fuscoceruleus ophthalmomaxillaris, is a dermal melanocytic hamartoma that presents as bluish hyperpigmentation along the first or second branches of the trigeminal nerve. Extra-cutaneous involvement has been reported, especially ocular involvement.

Nevus of Ota affects between 0.014%-0.034% of the Asian population. Clinical differential diagnoses include facial cafe-au-lait patch, spilus nevus, and acquired bilateral nevus of Ota-like macules. Previous treatment modalities, including cryotherapy and microsurgery, can be associated with scarring. In the last decade, the use of Q-switched lasers has revolutionized the treatment of this condition.

This review summarizes the clinical, histologic, and management aspects of this dermal melanocytic hamartoma.

Q-switched ruby laser in the treatment of nevus of Ota.

Yang HY, Lee CW, Ro YS, Yu HJ, Kim YT, Kim JH, Kim JH.

Department of Dermatology, Hanyang University, College of Medicine, Kuri Hospital, Kyunggido, Korea.

J Korean Med Sci 1996 Apr;11(2):165-70 Abstract quote

The purpose of this study is to evaluate the efficacy of Q-switched ruby laser in the treatment of nevus of Ota, a pigmented lesion on the face.

The Q-switched ruby laser has been shown to remove tattoos without scarring. With this in mind, the nevus of Ota with pigmented cells in the dermis could be effectively treated with Q-switched ruby laser. Eighty patients (19 men, and 61 women) with nevus of Ota on the face were enlisted to be tested in evaluating the efficiency of Q-switched ruby laser therapy. The age of patients ranged from 1 to 62 years. The energy fluence used varied from 6 to 8.5 J/cm2. Treatment intervals ranged from 4 to 16 weeks, and the number of treatment sessions varied between 1 to 9 visits. During a 2-year follow-up period, more than fifty percent removal of nevus-pigment was noted in 64 of the 80 patients. Transient hyperpigmentation was noted in 32 patients lasting for 2 to 6 months after treatment; transient hypopigmentation was seen in 3 cases which recovered within one year.No patients had permanent textural or pigmentary changes or scarring.

Q-switched ruby laser therapy appears to be an effective and safe modality for the treatment of nevus of Ota.

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Last Updated August 21, 2007

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