Ocular manifestations of facial port-wine stain, nevus of Ota, and phakomatosis pigmentovascularis in Asian patients.

BACKGROUND: Heightened intraocular pressure resulting in glaucoma and impaired vision is treatable if detected early. It is therefore necessary to identify populations at risk for glaucoma for regular screening visits. OBJECTIVE: To investigate the prevalence of glaucoma in patients with facial port-wine stains (PWSs), nevus of Ota, and phakomatosis pigmentovascularis (PPV) and to establish the association between facial vascular birthmarks and ocular complications. METHODS: This study is a retrospective chart review of 166 patients with facial PWS, PPV, and nevus of Ota over a 10-year period. RESULTS: Of the 166 cases, 76 patients were diagnosed with PWS, 83 with nevus of Ota, and 7 with PPV. The mean age of patients was 12.8 years, ranging from newborn to 63 years old. Fifteen patients were diagnosed with glaucoma. Of 15 patients, 11 presented with PWS, and 4 presented with both PWS and PPV. Of 83 patients with nevus of Ota, only 2 (2.4%) presented with increased ocular pressure. LIMITATIONS: The relatively short follow-up period is a limiting factor in this study. CONCLUSIONS: Early and periodic ophthalmic examinations in patients with PWS, PPV, and nevus of Ota are essential to minimizing the risk of developing glaucoma in these groups of patients.

Clinical profile and triggering factors for acquired, bilateral nevus of Ota-like macules.

BACKGROUND: Acquired, bilateral nevus of Ota-like macules (ABNOM) is one of the most common dermal melanocytoses. Although there are some literatures on ABNOM, its clinical features and etiopathogenetic factors have not been fully understood. OBJECTIVE: To determine the prevalence and characteristics of ABNOM among the Chinese patients. METHODS: A survey was carried out using the clinical examination and a questionnaire on 3212 first-time outpatients in our dermatology department, and 102 cases of ABNOM were subsequently enrolled. RESULTS: The outpatient prevalence of ABNOM was 3.18%, and the age of the onset was 27.2 years on average. They all presented as speckled macules on the face alone or coexisted with patchy lesions (17.7%) or a band-like pigmentation (1.0%). Unprecedentedly, we found the zygomatic arch, the infraorbital, the cheek and the parotid region can be involved, and 52.0% cases had sclera pigmentation. ABNOM commonly coexisted with the pigmented fungiform papillae of the tongue, the melasma, the acne, prementstrual syndrome (female) and breast cystic hyperplasia (female) with the rates of 33.3%, 20.6%, 26.5%, 47.0% and 43.0% separately. Triggering factors' investigation disclosed screen irradiation (47.1%), pregnancy (32.0%), cosmetics (29.4%), sensitive skin (22.6%), and positive family histories (21.6%) were highly related. CONCLUSIONS: Our study confirms that ABNOM is a relatively common disorder among adult Chinese's outpatients. It is commonly distributed over the malar, lateral forehead and temple's areas. The sclera pigmentation is another common finding that is overlooked in previous research. ABNOM is concomitant with melasma and some other disorders. Excessive sun exposure, hormonal disturbances and hereditary susceptibility are the main potential triggering factors of ABNOM.

Nevus of Ota: morphological patterns and distribution in 47 Yemeni cases.

BACKGROUND: Nevus of Ota is a rare pigmentary disorder characterized by melanocytic hamartoma of the skin along the distribution of trigeminal nerve branches. AIM: To study the morphological features of nevus of Ota, and their relation with facial regions and trigeminal branches areas. METHODS: Patients with nevus of Ota who attended Kuwait University Hospital dermatology clinic and a private dermatology clinic in Sana'a, Yemen, between January 2008 and December 2010, were clinically evaluated. The morphology of nevus of Ota was classified to homogenous, speckled or mixed. The distribution of the lesions was assessed based on both facial regions and areas supplied by the trigeminal nerve. RESULTS: Forty-seven patients with nevus of Ota (F = 38, M = 9) were evaluated. Eighty percent of patients were females. Mixed (homogenous and speckled) appearance was seen in 47% of all patients. Periocular region was the most commonly affected facial region (67%), and the maxillary area was the most commonly involved trigeminal branch area (94%). Forty-seven percent of cases had scleral pigmentation. Interesting features of nevus of Ota involving the nasal septum and crossing the midline of the face were noted. CONCLUSIONS: To the best of our knowledge, this is the first study that examines the distribution and morphology of nevus of Ota in Middle Eastern population. Including the morphological appearance of the nevus is practical and more clinically descriptive, and therefore should be integrated in the classification in future studies.

Nevus of Ota: clinical-ophthalmological findings / Nevo de Ota: achados clínicos e oftalmológicos

OBJECTIVE: To analyze the clinical and ophthalmological findings of patients with nevus of Ota. METHODS: Retrospective analysis of patients' charts with nevus of Ota. We registered the demographic data, location of the nevus and date of appearance, family history of similar spots, biomicroscopic, gonioscopic, tonometric, ophthalmoscopic and perimetric findings. RESULTS: We included 14 patients, six (43.0 percent) men and eight (57.0 percent) women, with a mean age of 21.7±17.5 years. Ten (71 percent) were mulatto, three (21.4 percent) white and one (7.1 percent) black. Twelve (85.7 percent) patients presented the spots at birth and two in puberty. Nine patients presented conjunctival and episcleral pigmentation in the right eye and five in the left eye. According to Tanino's classification, five (35.7 percent) nevi were class 1, eight (57.1 percent) class 2 and one (7.1 percent) class 3. Heterochromia iridis was found in eight (57.1 percent) patients. Anisocoria was present in three (21.4 percent) patients. Five (35.7 percent) patients presented a suspected glaucomatous cup disc ratio (≥0.7); six (42.9 percent) presented a cup disc ratio ≤ 0.5 and three (21.4 percent), no cup disc. We found two curious and remarkable findings: a nevus of Ota on the palate of one patient and other on the optic disc associated with a pigmentary mottling of the fundus in another patient. The pigmentary mottling of the fundus was also seen in four more eyes. CONCLUSIONS: The nevus of Ota was frequently present at birth, in mulattos, and classified as Tanino's class 1 and 2. Heterochromia iridis was a common finding. Anisocoria was present in a small percentage of eyes. No patient developed glaucoma or malignancy.

OBJETIVO: Analisar os achados clínico-oftalmológicos de portadores de nevo de Ota. MÉTODOS: Análise retrospectiva dos prontuários de pacientes com nevo de Ota. Foram registrados dados demográficos, localização do nevo e época do seu aparecimento, história familiar de manchas similares, olho acometido, achados ectoscópicos (classificação de Tanino), biomicroscópicos, fundoscópicos e campimétricos. RESULTADOS: Foram incluídos 14 pacientes, seis (47,0 por cento) homens e oito (53,0 por cento) mulheres com média de idade de 21,7±17,5 anos. Dez (71 por cento) pacientes eram feodérmicos, três (21,4 por cento) leucodérmicos e um (7,1 por cento) melanodérmico. Doze (85,7 por cento) pacientes apresentaram manchas ao nascimento e dois relataram seu surgimento após o nascimento. Três pacientes relataram manchas compatíveis com nevo de Ota em consanguíneos. A melanose conjuntivo-episcleral foi reconhecida no olho direito em cinco pacientes e em nove no olho esquerdo. Pela classificação de Tanino, cinco (35,7 por cento) dos nevos eram da classe 1, oito (57,1 por cento) da classe 2 e um (7,1 por cento) da classe 3. Presença de heterocromia da íris em oito (57,2 por cento) pacientes. Anisocoria ocorreu em três pacientes. Disco óptico suspeito de glaucoma (relação escavação/disco ≥ 0,7) foi observado em cinco pacientes (35.7 por cento); seis apresentaram escavação fisiológica e três não apresentavam escavação. Dois achados curiosos e raros: um nevo de Ota no palato de um paciente e outro no disco óptico de outro paciente associado com pigmentação difusa no polo posterior retiniano. Essa pigmentação foi também vista em mais quatro olhos. CONCLUSÃO: O nevo de Ota foi mais comumente presente ao nascimento, em feodérmicos e nas classes 1 e 2 de Tanino. Heterocromia iriana achado comum. Anisocoria foi diagnosticada num percentual pequeno de pacientes. Nenhum paciente desenvolveu glaucoma nem tumor maligno.

A population-based study of acquired bilateral nevus-of-Ota-like macules in Shanghai, China.

Acquired bilateral nevus-of-Ota-like macule (ABNOM) is a common skin dyspigmentation in Asian females. Although its clinical characteristics are well defined, its epidemiology and pathogenesis remain unclear. A large population-based cross-sectional study was conducted to determine the prevalence and risk factors of ABNOM. A total of 8,680 subjects (ages ranging from newborn to 99 years old; 54% female) were selected from urban areas in Shanghai, China, using a multistage sampling method. All participants (response rate=97%) were interviewed and examined for the presence of various pigmentary disorders by board-certified dermatologists. ABNOM was found in 2.5% of the study population (0.5% of males and 4.2% of females), and 90% of cases observed were female. In females, prevalence rose after the age of 15 years and sharply declined after the age of 50 years, with nearly half of the cases observed within ages 45-55 years (prevalence=8.5%). Age, contraceptive use, and sun exposure were independently associated with ABNOM. Women with ABNOM were less likely to have facial nevomelanocytic nevi and seborrheic keratoses. These findings indicate that sex hormone alteration and UV exposure may independently have an important role on the pathogenesis of ABNOM.

Glaucoma in oculodermal melanocytosis.

Oculodermal melanocytosis (ODM) is a developmental disorder of the embryonic neural crest involving virtually all of the structures of the eye derived from this tissue. The authors examined 194 previously unreported Thai patients with ODM. Elevated intraocular pressures (IOPs) developed in the ipsilateral eye of 15 patients at initial examination and in 5 others during follow-up, for a total of 10.3% of the patients. Three patients had congenital or late congenital glaucoma, 14 had ocular hypertension or open-angle glaucoma, and 3 had acute angle-closure glaucoma. Five of the patients with open angles had acute pressure rises accompanied by anterior uveitis. Melanocytic hyperpigmentation of the anterior chamber angle was extensive in all patients except those with acute angle-closure glaucoma. There was no consistent correlation between the extent or density of angle pigmentation and the development of elevated IOP. A larger cup-to-disc ratio in the involved eye, presumably congenital, was present in an additional 19 patients. Glaucoma is common in eyes with ODM and may develop at any age. Patients with ODM and initially normal IOP should be examined at regular intervals. Patients with open angles and acutely elevated IOP should be examined carefully for signs of anterior uveitis and treated medically.

Naevus fusco-caeruleus zygomaticus.

One hundred and ten cases of an unusual type of naevus, which we have called naevus fusco-caeruleus zygomaticus were studied. The naevus presents as a bilateral speckled discolouration of the skin of the face principally in the zygomatic region. The condition usually does not become apparent until the second decade of life, and is much commoner in females. No association with any other abnormalities was found. Light and electron microscopy of the speckles showed the presence of dermal melanocytes mostly in the upper dermis. A survey was also carried out to determine the prevalence of this naevus in the general population.