Blue Nevus Hidden within the Nevus of Ota.

A 3-year-old boy presented with bluish patch and scattered blue spots on the left side of his face. After several sessions of laser treatment, the azury patch in the periorbital area became even darker. Histopathology showed many bipolar, pigment-laden dendritic cells scattered in the papillary and upper reticular dermis. Immunohistochemically, these cells were positive for S100, SOX-10, melan-A, P16, and HMB-45. The positive rate of Ki-67 was less than 5%. Finally, the lesion was diagnosed with nevus of Ota concurrent with common blue nevus. Therefore, for cases of the nevus of Ota with poor response to laser treatment, the possible coexisting diseases should be suspected.

Efficacy and safety of Q-switched lasers for the treatment of naevus of Ota in children: a retrospective analysis.

To identify factors influencing the efficacy of Q-switched laser in the treatment of naevus of Ota in children and to compare the efficacy, safety, and recurrence rate between 1064 nm Q-switched Nd:YAG laser (QSNL) and 755 nm Q-switched alexandrite laser (QSAL). We retrospectively analysed 160 children with naevus of Ota who completed QSAL or QSNL laser treatment at our centre. Age at initial treatment (P = 0.004), colour of lesions (P = 0.025), and number of treatments (P = 0.002) were related to efficacy. Compared with patients aged 0-11 months at initial treatment, patients who started treatment at 1-3 years (OR adj = 0.47), 4-8 years (OR adj = 0.20), and 9-12 years (OR adj = 0.27) had inferior efficacy. The efficacy of brown-violet (OR adj = 2.67) and blue-violet lesions (OR adj = 2.51) was better than that of brown lesions. Moreover, patients who received 3-4 (OR adj = 2.83) or 5-6 (OR adj = 7.35) treatment sessions showed a better response than those who received 1-2 sessions. Additionally, as the age at initial treatment increased, the rate of complications increased from 2.0 to 14.3%, while the recurrence rate decreased from 8.2 to 0%. In addition, the complication rate increased with an increase in the number of treatments. There were no significant differences in clinical efficacy (P = 0.94), risk of complications (P = 0.752), or recurrence (P = 0.834) between QSAL and QSNL for treating naevus of Ota in children. QSAL and QSNL are equally effective for children's naevus of Ota, with low complications and recurrence rates. Younger age at initial treatment and a greater number of treatments are beneficial for efficacy, whereas brown lesions are a negative factor.

An update on ophthalmological perspectives in oculodermal melanocytosis (Nevus of Ota).

PURPOSE: To provide a review of the literature on oculodermal melanocytosis (ODM) with a focus on the diagnostic and therapeutic implications of multimodal imaging techniques in the management of ophthalmic complications. METHODS: The authors carried out a literature search on PubMed, Medline, and Scopus of English language articles published on ODM through August 2021. This review presents traditional and novel diagnostic methods in the diagnosis and follow-up of patients with particular emphasis on addressing the role of imaging in the management of the ophthalmic complications of the condition towards improving current practice patterns. RESULTS: ODM is a rare, prevalently unilateral, congenital condition that presents with brown or blue/gray flat asymptomatic lesions of the skin, mucosae, episclera/sclera, and uvea localized within the territory of distribution of the ophthalmic and mandibular branches of the trigeminal nerve. Glaucoma and predisposition to uveal melanoma are the main ophthalmic complications. Diagnosis and management are through comprehensive opthalmological examination and traditional imaging methods such as ultrasonography and fluorescein/indocyanine green angiography as pigmentation of the fundus can conceal subtle retinal and choroidal alterations. Anterior segment optical coherence tomography and ultrasound biomicroscopy are used to evaluate the anterior segment and the ciliary body in the presence of glaucoma or melanoma of the anterior uveal tract. Fundus autofluorescence and retinal pigment epithelium (RPE) alterations are of aid in the differential diagnosis between choroidal nevi and melanoma. Enhanced depth imaging spectral domain optical coherence tomography offers outstanding in vivo evaluation of the dimensions and details of tumors or nevi and surrounding choroidal tissues and small choroidal melanomas may show distortions of the retinal and sub-retinal profile, presence of intra and sub-retinal fluid, abnormalities of the RPE, and compression of the choriocapillaris. CONCLUSIONS: Novel multimodal imaging techniques are significant in the diagnosis and management of the ophthalmic complications of ODM. Fundus autofluorescence and enhanced depth spectral domain optical coherence tomography have adjunctive value in the detection of early-stage melanoma and differential diagnosis between nevi and melanoma. Awareness of current and emerging imaging techniques can propagate improved standardized definition and assessment of the complications of ODM.

Nevus of Ota on the auricle successfully treated with Q-switched ruby laser.

Nevus of Ota is a dermal melanocytosis that consists of blue-brown spots, patches and plaques along the distribution of the first and second branches of trigeminal nerve. The efficacy of Q-switched ruby laser treatment against nevus of Ota on dark skin has not been described. The present case, a 2-month-old Indonesian girl, showed rare auricular involvement. Because ear has complicated steric structure, whose skin is sensitive and thin, pain and inflammatory reaction are inevitable. We discussed the difficulty of laser treatments on auricular lesions.

GNA11 Mutation in an Intracranial Melanocytoma with Orbital Involvement and Nevus of Ota.

The prognostic value of mutations in G-protein genes GNAQ and GNA11 in patients with intracranial and orbital melanocytomas is unknown. The authors present a case of GNA11 mutation (GNA11Q209L) in a 32-year-old male suffering from a meningeal melanocytoma with orbital involvement and ipsilateral Nevus of Ota. The patient underwent gamma knife stereotactic radiosurgery without biopsy and later partial transcranial resection of the melanocytic tumor that was subject to immunohistochemical and molecular analysis. A 50-gene next-generation sequencing panel revealed a 626A>T mutation in the GNA11 gene. One year later, intracranial extension of the melanocytoma necessitated a ventriculoperitoneal shunt and immunotherapy. Future work is needed to determine how GNA11 mutations in melanocytomas influence prognosis and monitoring strategies.

Efficacy and safety of picosecond 755-nm alexandrite laser for treatment of nevus of Ota in Taiwanese children: A retrospective study.

OBJECTIVES: To evaluate the efficacy and safety of picosecond 755-nm alexandrite laser in the treatment of nevus of Ota in children. MATERIALS AND METHODS: A retrospective study was conducted by reviewing medical charts and photographs of 86 Taiwanese children with various types of nevus of Ota between January 2017 and September 2020. Picosecond 755-nm alexandrite laser therapy was used to treat pigmentary lesions. Percent clearance of lesions during treatment and the treatment time required to achieve 95%-100% clearance were determined. RESULTS: According to Tanino's classification or Peking University Medical College Hospital (PUMCH) classification of nevus of Ota, most patients belonged to Tanino's Type II (32%) and Type III (38%) or PUMCH Type IIb (33%) and Type IIIb (26%), which indicated that the nevus was mainly distributed in the forehead, upper and lower eyelid, zygomatic, cheek, and temple regions. After treatment with picosecond 755-nm alexandrite laser, 96.5% of the patients achieved 95%-100% clearance with an average of 4.3 treatment sessions. The earlier onset of lesions (before 5 months of age) and the darker Fitzpatrick skin types (type IV vs. type III) significantly increased the number of treatments required to achieve clear response, while sex, age at first treatment, Tanino's classification of nevus, and color of nevus had no significant effect. Posttreatment hypopigmentation or hyperpigmentation was transient and resolved within 6 months. No serious response of the skin was evident. CONCLUSION: Picosecond 755-nm alexandrite laser treatment of nevus of Ota in children was safe and effective. The treatment was well-tolerated, and only a few transient, minor side effects occurred.

Naevus of Ota: clinical characteristics and proposal for a new ocular classification and grading system.

INTRODUCTION: Naevus of Ota is a congenital condition that may involve the skin, eyeball and even intracranial structures usually in the distribution of the ophthalmic and maxillary divisions of the trigeminal cranial nerve. The purpose of this study was to summarise our experience with the ocular clinical presentation, imaging, outcome, treatment of complications and to offer a new classification of patients with naevus of Ota. METHODS: We retrospectively reviewed the patients' medical records and the following parameters were retrieved and analysed: demographics, clinical presentation complications and treatment of complications. Imaging characteristics of patients with naevus of Ota were compared with images from the same period of time of 57 age-matched and gender-matched patients without naevus of Ota (control group). RESULTS: The series was composed of 40 patients (18 males, 22 females) whose mean age at diagnosis was 35.27 years (range 0.5-77 years). Thirty-three patients (82.5%) were type I naevus of Ota according to the Tanino classification, three patients (7.5%) were type II, one patient (2.5%) was type III and three patient (7.5%) were type IV (bilateral naevus of Ota). We further classified all cases in according to the ocular involvement extent. Three patients developed malignant transformation to choroidal melanoma and four patients developed glaucoma. CONCLUSIONS: In this study, a new clinical classification based on the involved ocular component and extent of the involvement (in quadrants) of the globe is suggested first. Further studies are needed to assess whether our clinical ocular classification can assist in identifying patients at risk for developing glaucoma and malignant transformation.

Comparing the efficacy and safety of Q-switched and picosecond lasers in the treatment of nevus of Ota: a systematic review and meta-analysis.

Nevus of Ota is cosmetically burdensome and often prompts patients to seek treatment. Lasers are commonly used in removing these lesions; however, no systemic analysis has been conducted to support a gold standard laser. To conduct a meta-analysis of the efficacy and safety of Q-switched Nd:YAG lasers (QSNL), Q-switched ruby lasers (QSRL), Q-switched alexandrite lasers (QSAL), and picosecond alexandrite lasers (PSAL) in removing nevus of Ota. Inclusion criteria were nevus of Ota patients treated with QSNL, QSRL, QSAL, or PSAL and documentation of percent clearance and the rate of at least one adverse event. Articles in English, Chinese, or Japanese were included. The prespecified outcome measures were efficacy (percent clearance) and safety (rates of hyperpigmentation, hypopigmentation, scarring, and recurrence). The review included 57 studies and 13,417 patients. The pooled success rate was 64% for QSNL (95% CI 52-76%), 54% for QSRL (95% CI 39-69%), 58% for QSAL (95% CI 44-72%), and 100% for PSAL (95% CI 98-102%). The pooled adverse event rate was 5% for QSNL (95% CI 4-6%), 14% for QSRL (95% CI 9-19%), 9% for QSAL (95% CI 6-12%), and 44% (95% CI 31-57%) for PSAL. QSNL has the most evidence for effectively and safely treating nevus of Ota. PSAL potentially has a superior efficacy; however, further studies are needed to elucidate its side effect profile when treating nevus of Ota.

Primary acquired melanosis (PAM) without atypia/WHO low-grade conjunctival melanocytic intraepithelial lesion over areas of oculodermal melanocytosis.

An elderly white man with a history of left oculodermal melanocytosis presented with new onset brown pigmentation of the left bulbar and inferior tarsal conjunctiva. The bulbar conjunctival pigmentation was at the level of the conjunctival epithelium and was overlying areas of typical slate-grey scleral pigmentation characteristic of oculodermal melanocytosis. Both areas of new pigmentation were biopsied. The bulbar conjunctiva revealed primary acquired melanosis (PAM) without atypia with increased melanin production and the tarsal conjunctival biopsy showed PAM without atypia sine pigmentio overlying areas of substantia propria spindle-shaped heavily pigmented melanocytes of oculodermal melanocytosis. The case report examines the relationship between the epithelial and substantia propria melanocytes and correlates the findings with what is known about this association from the dermatopathology literature.

In vivo reflectance confocal microscopy for evaluating common facial hyperpigmentation.

BACKGROUND: In clinical settings, atypical facial hyperpigmentation such as nevus of Ota, acquired bilateral nevus of Ota-like macules (ABNOM), melasma, and café-au-lait spots are often missed and misdiagnosed. Summarizing in vivo reflectance confocal microscopy (RCM) features of the hyperpigmentation is helpful in the diagnosis of ambiguous lesions. METHODS: We recruited 196 patients referred for unequivocal facial hyperpigmentation, including 55 patients with nevus of Ota, 45 patients with ABNOM, 62 patients with melasma, and 34 patients with café-au-lait spots. The RCM images were evaluated at the epidermis, the dermis-epidermis junction (DEJ), and the upper papillary dermis from both hyperpigmented patches and normal skin. RESULTS: In the superficial and middle dermis, 41 of 55 patients (74.5%) with nevus of Ota were characterized by a cord-like or lumpy structure between the collagen fibers. And there was no melanin deposition detected in the dermis in 14 of 55 (25.5%) patients. In ABNOM, 37 of 45 (82.2%) patients were characterized by a cord-like or lumpy structure in the superficial dermis and 8 of 45 patients (17.8%) was no melanin deposition detected in the dermis. The epidermis was no difference between nevus of Ota, ABNOM, and the normal skin. Melasma was detected increased cobblestone pattern in the epidermis of all patients, branching architecture in 21 of 62 patients (33.9%), and focally aggregated round to triangular cells in the upper dermis of 18 of 62 patients (29.0%). In all patients with afé-au-lait spots, increased cobblestone pattern in the epidermis and regular and increased density of ringed pattern in the DEJ were visualized. CONCLUSIONS: Our findings indicate that RCM may be useful in the auxiliary diagnosis of nevus of Ota, ABNOM, melasma, and café-au-lait spots.

Successful treatment of a patient with multicolored nevus of Ota using a combination of 1064 and 532 nm Q-switched Nd:YAG lasers.

Nevus of Ota (NO) is a hamartoma of dermal melanocytes usually presents as unilateral blue, gray or brown macules or patches. It can impose a high burden of cosmetic and psychological disturbances in affected individuals. Q-Switched lasers appear to be an effective treatment for this kind of dermal melanocytosis. Multicolored Ota nevus is a rare variant of this kind of nevus and its treatment may be more challenging compared with unicolor lesions. Herein we report a 21-year-old woman with a multicolored nevus of Ota (blue and brown), which showed a dramatic response to a combination of 1,064 nm and 532 nm Q-Switched Nd:YAG lasers. We also discuss different aspects of the Q-switched laser application of Ota nevus treatment. We also focus on laser combination therapy to treat the nevus of Ota.

Therapeutic options for management of Hori's nevus: A systematic review.

Hori's nevus is a pigmentation disorder reported mainly in middle-aged Asian women. There is no systematic review addressing its pharmacotherapy. The population for our systematic review was patients with a clinical/histological diagnosis of Hori's nevus (both sex, any age group). We screened five literature databases using relevant keywords. All RCTs, observational studies and case series mentioning at least one intervention and outcome of that intervention were included. Nineteen studies were included in the final systematic review from total 680 identified nonduplicate records. Different forms of laser (alexandrite laser [QSAL and PSAL], Nd:YAG laser [QSNYL high fluence, low fluence, 532 followed by 1064 nm], Er: YAG and Nd:YAG combination, ruby laser [QSRL], CO2 laser followed by QSRL) and dermabrasion were found to be useful in treatment of Hori' nevus. Among alexandrite lasers, PSAL is more efficacious and safe than QSAL. In case of high fluence QSNYL, hyperpigmentation rate is quite high while low fluence QSNYL requires more number of treatment sessions. The combined 1064 nm + 532 nm protocol is better in terms of efficacy and safety. Er:YAG + Nd:YAG combination have similar efficacy and added advantage of synergistic action and no adverse event.

An extensive nevus of Ota with oro-buccal involvement: a case review and proposal of a new classification system.

Nevus of Ota (NO) also known as nevus fuscoceruleus ophthalmomaxillaris or oculodermal melanocytosis is a rare hamartoma. It is usually characterized by unilateral, patchy and speckled, blue/gray or brown macules on the forehead and periocular area. Here, we report a rare case of NO with oro-buccal involvement, which is an unusual condition. The patient was presented due to the rarity of oro-buccal involvement, extensive and remarkable expression of the lesions, and in order to propose a more comprehensive and neuroanatomy based classification system.

Clinical and genetic characteristics of nevus of Ota with choroidal melanoma in Chinese.

Purpose: The aim of the present study is to report the clinical and genetic characteristics of nevus of Ota with choroidal melanoma in Chinese patients. Patients and Methods: Patients with nevus of Ota with choroidal melanoma were identified by searching the computerized database and patient medical records of Beijing Shijitan Hospital and Shaanxi Yulin Tradition Chinese Medicine Hospital. The patients (2 men and 1 woman; mean age, 52 years; age range, 52­57 years) were all treated by enucleation or local endoresection, and choroidal melanoma was confirmed by pathologic examination. Results: The patients (2 men and 1 woman; mean age, 52 years; age range, 52­57 years) were all treated by enucleation or local endoresection, and choroidal melanoma was confirmed by pathologic examination. The study found that patients with nevus of Ota had higher risk for malignant melanoma. Furthermore, we found two suspicious gene mutations involving FAM111B and DSC2, that might contribute to the etiology of the disease. Conclusions: The results indicate that patients with nevus of Ota should undergo regular ophthalmological observation and be aware of the potential for malignancy.

Nevus of Ota - an intraoral presentation: a case report.

BACKGROUND: Nevus of Ota or "oculodermal melanocytosis" is a rare congenital hamartoma of dermal melanocytes causing a blue-gray hyperpigmentation of the eye and surrounding structures. The condition, originally described by Ota and Tanino in 1939, mainly affects the ophthalmic and maxillary divisions of the trigeminal nerve. We describe the first reported case of unilateral oculodermal melanocytosis in a Caucasian woman with oral buccal mucosal involvement. Oral involvement of nevus of Ota is very rare. CASE PRESENTATION: A 48-year-old Caucasian woman was referred by the dermatology division to the oral medicine department at the University of Liverpool School of Dentistry with new-onset oral pigmentation to the left buccal mucosa. The patient had a previous diagnosis of oculodermal nevus. CONCLUSION: An incisional biopsy of the left buccal mucosa was completed. The report stated that histological and immunohistochemical features were in keeping with a blue nevus, but within the context of the preexisting occulodermal pigmentation, a diagnosis of oculodermal melanocytosis, also known as "nevus of Ota," was made. The patient will be kept under review in the oral medicine department because the progression of the lesion on the left buccal mucosa requires active monitoring owing to the potential for malignant change. The patient also requires regular review in the dermatology and ophthalmology divisions.

Meningeal Melanocytoma Associated with Nevus of Ota: Analysis of Twelve Reported Cases.

BACKGROUND: Primary melanocytic neoplasms (PMNs) are rare neoplasms, especially within the central nervous system. Meningeal melanocytomas, a subtype of PMN, are even rarer. Nevus of Ota results from the incomplete migration of melanocytes from the neural crest. Synchronous nevus of Ota and meningeal melanocytoma are infrequently encountered in clinical practice. OBJECTIVE: To evaluate and elucidate 12 cases of synchronous meningeal melanocytoma and nevus of Ota, thereby improving the understanding of the relationship between these 2 diseases. METHODS: We reviewed cases and searched the English-language literature from the PubMed database and collected clinical parameters of 12 cases of synchronously occurring nevus of Ota and meningeal melanocytoma. RESULTS: Among the 12 cases, 90.90% and 91.66% of the lesions were located ipsilaterally and supratentorially, respectively. CONCLUSIONS: Our findings indicated a trend for both types of lesion to be located ipsilaterally and supratentorially. When a patient with nevus of Ota is found to harbor an intracranial neoplasm, the most likely diagnosis is PMN.

Oculodermal Melanocytosis: Nevus of Ota in a Dog.

This report describes the clinical presentation, diagnosis, and histopathologic features of oculodermal melanocytosis in a young dog. A 3-year-old male neutered Labrador Retriever presented with conjunctival and scleral hyperpigmentation of the right eye, with concurrent ipsilateral cutaneous hyperpigmentation involving the right side of the face. Initial skin and conjunctival biopsies revealed an accumulation of histologically benign melanocytes within the dermis and conjunctival stroma, respectively. Enucleation was elected 19 months later by the referring veterinarian due to the progression of ocular pigmentation with concurrent marked corneal lipidosis and the suspicion of a scleral mass. On gross and histopathologic examination of the globe, there was marked panuveal melanocytosis with extension into the sclera, bulbar conjunctiva, and connective tissue surrounding the optic nerve, as well as sharply demarcated ipsilateral hyperpigmentation of the facial skin. The findings are characteristic of oculodermal melanocytosis (nevus of Ota), a dermal melanocytic hamartoma presenting as cutaneous facial hyperpigmentation that corresponds to the distribution of the ophthalmic and maxillary branches of the trigeminal nerve, often with ipsilateral ocular involvement.