ABSTRACT
Background: Acquired dermal melanocytosis is a heterogenous group of hyperpigmented lesioins which predominantly involve the face. Aim: The aim of this study was to study the clinical presentation and histopathology of cases with extra-facial acquired dermal melanocytosis. Methods: Retrospective record analysis was performed between May 2016 to August 2019 to retrieve cases of extra-facial acquired dermal melanocytosis seen at the out-patient department of dermatology at the All India Institute of Medical Sciences, Jodhpur. Consecutive cases with histopathologically proven diagnosis of acquired dermal melanocytosis were included. Documentation of variation in clinical presentation and histopathologic findings was done in light of the existing literature. Results: Overall, four cases of extra-facial acquired dermal melanocyosis (female:male = 1:3) were seen during the study period. The lone case on head and neck involved the ear lobule and peri-auricular area. The other three cases had involvement of the hand. The histopathology confirmed the diagnosis of dermal melanocytosis but revealed peculiar findings of angiotropic melanocytes and dilated capillaries. Limitations: Small sample size and lack of comparison with perilesional normal skin were the limitations of this study. Conclusion: The findings of angiotropic melanocytes may be unique to extra-facial acquired dermal melanocytosis. This might indicate interaction between dermal melanocytes and capillary endothelial cells. This finding along with dermal capillary ectasia may indicate a possible role for vascular lasers in the management of these disorders
ABSTRACT
Aims: To describe Congenital Ocular Melanocytosis.Presentation of Case: LPC, 7 years old, male, brown, with no previous comorbidities, was taken to the ophthalmology outpatient clinic of the Hospital Universit醨io Ant鬾io Pedro, Brazil by parents who alleged the presence of bluish-looking lesions in the sclera of the child's right eye since birth.Discussion: Congenital Ocular Melanocytosis is a rare pathology characterized by an increase in the number, size and pigmentation of melanocytes. Its pathophysiological picture is unknown, but it is believed to be due to an alteration in the migration of melanocytes from the neural crest to the epidermis during the embryonic process. This condition can be complicated by glaucoma and uveal melanoma. Gonioscopy is essential in these cases to assess whether there is pigmentation of the trabeculae, so that the propaedeutics of investigation of glaucoma becomes essential in these patients, since 10% of cases can complicate this condition.Conclusions: Congenital Ocular Melanocytosis early in life and the importance of monitoring these patients should be emphasized. Comprehensive tests are important for early detection and treatment, in order to improve the prognosis and avoid more severe consequences than what can happen from melanocytosis.
ABSTRACT
We report a case of trifocal choroidal melanoma (three separate tumors) in a 48-year-old Caucasian female who had been followed for oculodermal melanocytosis since childhood. At presentation, no tumor was present and annual examination was advised. Seventeen years later, three choroidal melanocytic lesions were detected in the right eye. Growth of each was documented, enucleation was performed, and histopathology revealed three independent choroidal melanomas. The patient developed extensive liver and bone metastases and subsequently died. Oculodermal melanocytosis is a risk factor for the development of uveal melanoma and a potential marker for worse prognosis. Careful long-term follow-up is required.
ABSTRACT
A 51-year-old Asian Indian male with right oculodermal melanocytosis presented with a blurred visual acuity of 20/40. Upon fundus examination, he was discovered to have two independent pigmented choroidal melanomas in the temporal juxtapapillary region and inferiorly, in the region of trace clinical sectoral choroidal melanocytosis. The patient underwent enucleation, and on histopathology the two tumors were found to be in the area of choroidal melanocytosis, in continuity with each other. Multifocal choroidal melanoma is an exceedingly rare diagnosis. Ocular melanocytosis is a known predisposing factor for both unifocal and multifocal melanomas. Lifelong monitoring for uveal melanomas must be done in all patients with ocular melanocytosis.
ABSTRACT
Resumen Introducción: Las melanocitosis dérmicas son un grupo de enfermedades dermatológicas pigmentarias asociadas con la proliferación melanocítica. Se clasifican con base en su número y localización profunda a nivel de la dermis; pueden ser congénitas o adquiridas. Caso clínico: Paciente de sexo masculino de 11 años de edad, sin antecedentes de importancia para el padecimiento actual. Inicio con mácula oscura en la palma de la mano izquierda hace 5 años, asintomática, de crecimiento paulatino. A la exploración física, se detectó dermatosis que afectaba la palma izquierda, cara palmar de las falanges proximales del tercer y cuarto dedos, caracterizada por la presencia de mácula grisácea negruzca, bordes difusos e irregulares, no infiltrada ni indurada. En la dermatoscopia se detectó un patrón de pigmento de tono gris acero con áreas de color café, y con evidencia de puntos blanquecinos dentro de estas. En la histopatología se identificaron células fusiformes, con núcleo grande y la presencia de pigmento melánico en su interior, con distribución perivascular y entremezclados con las fibras de colágeno en la dermis superficial y media. Con base en las características clínicas e histopatológicas de la lesión, se concluyó melanocitosis dérmica adquirida de la mano como el diagnóstico definitivo. Conclusiones: Los reportes de casos de melanocitosis dérmica adquiridas atípicas son infrecuentes. La melanocitosis dérmica de la mano es una variante de estas enfermedades, de la que existen menos de 10 casos. Se presenta el primer caso reportado en Latinoamérica hasta el momento, con el objetivo de ampliar el conocimiento de sus características clínico-histológicas y dermatoscópicas.
Abstract Background: Dermal melanocytosis is a group of pigmentary dermatological diseases associated with melanocytic proliferation, which are classified based on their number and depth at the level of the dermis; they may be congenital or acquired. Case report: An 11-year-old male patient with no history of importance for the current condition started 5 years ago with a dark macula in the left hand palm, which was asymptomatic but grew gradually. On physical examination, dermatoses affecting the left palm, palmar face of proximal phalanges of the third, fourth and fifth fingers, characterized by the presence of blackish greyish macula, diffuse and irregular edges, not infiltrated or indurated were detected. The dermatoscopy identified a pattern of pigment with a greyish-brown tone with brown areas, showing whitish spots inside. In the histopathology, the presence of spindle cells was observed in the superficial and middle dermis, with a large nucleus and the presence of a melanic pigment inside, with perivascular distribution and intermingled with the collagen fibres. Based on the clinical characteristics and the histopathological findings, acquired dermal melanocytosis of the hand was concluded as the final diagnosis. Conclusions: Case reports of atypical acquired dermal melanocytosis are infrequent. Dermal melanocytosis of the hand is a rare variant of these diseases, of which less than 10 cases have been reported. At present, this case of dermal melanocytosis is the first reported in Latin America with the aim to extend the knowledge of its clinical-histological and dermatoscopic characteristics.
Subject(s)
Child , Humans , Male , Pigmentation Disorders/pathology , Cell Proliferation , Hand Dermatoses/pathology , Melanocytes/pathology , DermoscopyABSTRACT
Introducción: la melanocitosis dérmica incluye un espectro de lesiones de piel que abarca la mancha mongólica, entre otras lesiones. Las enfermedades lisosomales son afecciones de base genética que se caracterizan por la acumulación de metabolitos insolubles parciamente degradados en los compartimentos lisosomales, debido a una determinada deficiencia enzimática. Las deficiencias de b-galactosidasa y de a-L-iduronidasa provocan la gangliosidosis GM1 y la mucopolisacaridosis tipo I respectivamente, ambas presentando similitudes en su presentación clínica. La asociación de la melanocitosis dérmica con las enfermedades lisosomales es poco común y mal entendida. Objetivo: reportar dos pacientes con esta rara asociación. Casos clínicos: dos varones de 3 y 9 meses sin antecedentes prenatales ni perinatales a destacar y antecedentes de infecciones respiratorias reiteradas. Se presentaron con retraso del desarrollo, hipotonía central y trastorno deglutorio. Al examen se constató hepatomegalia, fascies tosca y melanosis dérmica extensa. Los estudios permitieron diagnosticar al paciente de 3 meses mucopolisacaridosis Tipo I y al de 9 meses gangliosidosis GM1. Discusión: no se conoce exactamente la causa de esta asociación. Se plantea que sería el resultado de la acumulación de gangliósidos y heparán sulfato que estimularían al receptor del factor de crecimiento neuronal de tipo tirosinquinasa, deteniendo la migración de los melanocitos en la dermis. Por lo tanto la melanosis dérmica aberrante, en el contexto clínico adecuado, puede ser un signo que facilite el diagnóstico de una enfermedad lisosomal subyacente.
Introduction: dermal melanocytosis includes a spectrum of skin lesions, mongolian spots being one of them. Lysosomal storage diseases are characterized by the accumulation of partially degraded insoluble metabolites in lysosomal compartments due to enzyme deficiency. Deficiency in b-galactosidosisis is the cause of GM1 gangliosidosis and deficiency in a-L-iduronidasa of mucopolysaccharidosis type I. Both have similar clinical presentations. Association of dermal melanocytosis and lysosomal storage diseases is uncommon and misunderstood. Objective: to report the case of two patients with this rare association. Clinical cases: the study presents two boys, 3 and 9 months old, with no remarkable family, pregnancy or delivery history. Both had repeated respiratory tract infections. They presented with developmental delay, central hypotonia and swallowing disorder. Upon clinical examination they showed hepatomegaly, coarse facies and extensive dermal melanocytosis. They were diagnosed with GM1 gangliosidosis and mucopolysaccharidosis type I. Discussion: the cause of this association is not well known. It is hypothesized that accumulation of gangliosides and heparan sulfates stimulates tyrosine-kinase neuronal growth factor receptor, stopping dermal melanocytosis migration. Therefore extensive dermal melanocytosis, in an appropriate clinical setting, may contribute to diagnosing lysosomal storage diseases.
Subject(s)
Humans , Male , Infant , Skin Neoplasms , Lysosomal Storage Diseases/complications , Gangliosidosis, GM1/diagnosis , Mucopolysaccharidosis I/diagnosis , Mongolian Spot/etiologyABSTRACT
Nevus of Ota is a hamartoma of dermal melanocytes presenting as blue hyperpigmented patches on the face usually within the distribution of ophthalmic and maxillary branches of the trigeminal nerve.[1] A case of giant nevus of Ota involving all three branches of the trigeminal nerve is presented.
ABSTRACT
Background: Spectral domain enhanced depth imaging optical coherence tomography (EDI‑OCT) can provide anatomic localization of intraocular tumors. Aims: The aim was to identify topographical and intrinsic patterns of choroidal tumors on EDI‑OCT. Settings and Design: Retrospective review. Materials and Methods: Analysis of published reports and personal observations using office based EDI‑OCT. Results: Using EDI‑OCT, choroidal nevus displayed a smooth, dome‑shaped topography with overlying retinal pigment epithelium alterations, drusen, and occasional subretinal cleft demonstrating photoreceptor loss. Small choroidal melanoma showed smooth, moderately dome‑shaped topography, commonly with overlying shallow subretinal fluid that often depicted “shaggy” photoreceptors. Choroidal metastasis showed a minimally “lumpy, bumpy” surface topography and with overlying subretinal fluid and shaggy photoreceptors. Choroidal hemangioma showed a smooth, dome‑shaped topography, with expansion of the affected small, medium, and large choroidal vessels. Choroidal lymphoma showed varying topography with increasing tumor thickness as “flat, rippled, or undulating (seasick)” surface. Choroidal osteoma displayed a smooth undulating surface with visible intralesional horizontal lines suggestive of bone lamellae and occasional horizontal and vertical tubules with intralesional “spongy” flecks. Choroidal melanocytosis appeared as uniformly thickened choroid with increased stromal density surrounding the normal choroidal vascular structures. Conclusions: Enhanced depth imaging‑OCT can depict characteristic patterns that are suggestive of various choroidal tumors.
ABSTRACT
BACKGROUND:Mongolian spots (MS) are congenital birthmarks seen most commonly over the lumbosacralarea. They are bluish-green to black in color and oval to irregular in shape. Dermal pigmentationappears gray, greyish-blue or greyish black because these colors have a shorter wavelength andare reflected to the skin surface. The amount of melanin in the dermal melanocytes, the numberof dermal melanocytes and their depth in the dermis are also important determinants of color. TheMongolian blue spots often fade in a few years and are almost always gone by adolescence.AIM:The aim of this study was to determine the frequency and characteristics of Mongolian spots in 1-10-years-old children.MATERIALS AND METHODS:The study was carried out on 1000 children who were lived in Ulaanbaatar during 2013-2014.Children were carefully examined for the presence of Mongolian spots, including the hairy skin,during the systematic physical examination. If a spot was observed, localization, size, color andshape were recorded. Statistical analysis was done using SPSS-21.RESULT:96.8% (95%CI 95.7-97.8) of the children’s was residents of Ulaanbaatar city, 49.2% [95%CI 46.1-52.5] of boys. The color of the Mongolian spots varied from pale blue to grayish blue. The mostfrequently involved sites were the lumbosacral areas. There was a disappearance in the incidenceof Mongolian spots with advancing age.CONCLUSION:The study shows that prevalence of blue spot among children aged 1-10 years old decreased withage and blue spot tended to disappear after the age of 10.
ABSTRACT
Vitiligo is one of the most common pigmentary skin disorders; it is characterized by circumscribed depigmented macules due to the destruction of melanocytes. Although the etiology of vitiligo has not been fully elucidated, multiple factors including autoimmune and oxidative stress have been implicated in the pathogenesis of vitiligo. In contrast, dermal melanocytosis is histologically characterized by the presence of dermal melanocytes. It has been described that there are ectopic dermal melanocytes, which have failed to reach their proper location. A literature search revealed very few reports of patients with vitiligo developing vitiligo within dermal melanocytosis. Here, we report two cases of patients with vitiligo that occurred at pre-existing sites of dermal pigmented lesions. The histopathology showed the loss of epidermal melanocytes in spite of the existence of melanocytes in the dermis. There was no significant infiltration of inflammatory cells in the dermis. These cases illustrate unknown environmental factors as well as heterogeneity.
Subject(s)
Humans , Dermis , Melanocytes , Oxidative Stress , Population Characteristics , Skin , VitiligoABSTRACT
Dermal melanocytosis is most commonly found in the skin of Asians and other darkly pigmented populations. It is histologically characterized by the presence of ectopic melanocytes in the dermis. Mongolian spots, nevus of Ota, nevus of Ito and blue nevus are the most common types, and these are usually present at birth or in early childhood. However, acquired dermal melanocytoses that appear in adult life are rare. A 65 year-old female had taken imatinib mesylate to treat gastrointestinal stomach tumor. Few months later, brownish or slate-bluish pigmented patches appeared on her face, supraclavicular, and scapular area. The skin biopsy specimen, taken from the forehead, revealed scattered, pigmented, spindle-shaped cells, and dendritic cells containing brown pigment in the dermis. Herein, we report a case of acquired dermal melanocytosis induced by imatinib mesylate.
Subject(s)
Adult , Female , Humans , Asian People , Benzamides , Biopsy , Dendritic Cells , Dermis , Forehead , Melanocytes , Mesylates , Mongolian Spot , Nevus , Nevus of Ota , Nevus, Blue , Parturition , Piperazines , Pyrimidines , Skin , Stomach , Imatinib MesylateABSTRACT
Dermal melanocytosis is characterized by the presence of ectopic melanocytes in the dermis. The most common forms include the Mongolian spot, blue nevus, nevus of Ota, and nevus of Ito. Some types of dermal melanocytosis do not fit into any of these morphologic categories, however. Our case demonstrated an extensive amount of uniform deep blue patches of nevi with unilateral distribution on the left face, neck, chest, shoulder, and back. On histopathologic examination, a number of elongated melanocytes scattered throughout the dermis were found. We herein report a case of congenital unilateral dermal melanocytosis.
Subject(s)
Dermis , Melanocytes , Mongolian Spot , Neck , Nevus , Nevus of Ota , Nevus, Blue , Shoulder , ThoraxABSTRACT
Dermal melanocytosis, which is histologically characterized by the presence of dermal melanocytes, is most commonly found in Asians and other darkskinned people. It is observed in various congenital conditions such as the nevus of Ota, the nevus of Ito, the Mongolian spot and the blue nevus, and typically appears at birth or in early childhood. However, several cases that have appeared in adult life have been reported as acquired dermal melanocytosis. Herein, we report a unique case of acquired dermal melanocytosis diffusely affecting the left side of the chest of a 45-year old female. Additionally, a review of Korean literature regarding reported cases of unusual acquired dermal melanocytosis will be presented.
Subject(s)
Adult , Female , Humans , Asian People , Melanocytes , Mongolian Spot , Nevus , Nevus of Ota , Nevus, Blue , Parturition , ThoraxABSTRACT
Dermal melanocytosis includes a variety of congenital and acquired conditions characterized by a sparse population of intradermal dendritic, variably pigmented, spindle-shaped melanocytes. Mongolian spot, Ota's and Ito's nevi are usually present at birth or appear in early childhood. But, several types of acquired dermal melanocytosis that usually appear in adults have been reported. A 47-year-old woman presented with asymptomatic, multiple, symmetric, brownish to black colored macules on the nasal ala, both palms and both feet dorsum that had been there for 10 years. Histopathologic findings showed that some scattered spindle-shaped cells containing melanin pigment in the upper dermis were positive for Fontana-Masson stain and these cells were positive for S-100 protein and MART-1.
Subject(s)
Adult , Female , Humans , Middle Aged , Dermis , Foot , Hand , Melanins , Melanocytes , Mongolian Spot , Nevus , Parturition , S100 Proteins , Silver NitrateABSTRACT
Dermal melanocytosis includes a variety of congenital and acquired conditions characterized by a sparse population of intradermal dendritic, variably pigmented, spindle-shaped melanocytes. Mongolian spot, Ota's and Ito's nevi are usually present at birth or appear in early childhood. But, several types of acquired dermal melanocytosis that usually appear in adults have been reported. A 47-year-old woman presented with asymptomatic, multiple, symmetric, brownish to black colored macules on the nasal ala, both palms and both feet dorsum that had been there for 10 years. Histopathologic findings showed that some scattered spindle-shaped cells containing melanin pigment in the upper dermis were positive for Fontana-Masson stain and these cells were positive for S-100 protein and MART-1.
Subject(s)
Adult , Female , Humans , Middle Aged , Dermis , Foot , Hand , Melanins , Melanocytes , Mongolian Spot , Nevus , Parturition , S100 Proteins , Silver NitrateABSTRACT
The dermal melanocytoses include a variety of pigment lesions that are formed from the aberrant development of the neural crest-derived melanocytes as they migrate through the dermis during embryogenesis. Neurocristic cutaneous hamartomas is one type of the dermal melanocytoses, which contain dermal melanocytic, neurosustentacular and fibrogenic components and can undergo malignant transformation. We describe a case of dermal melanocytosis showing features of neurocristic cutaneous hamartoma.
Subject(s)
Female , Pregnancy , Dermis , Embryonic Development , Hamartoma , MelanocytesABSTRACT
Dermal melanocytosis is histologically characterized by the presence of ectopic melanocytes in the dermis. Mongolian spots, nevus of Ota, nevus of Ito and blue nevus are the most common types and these are usually present at birth or in early childhood. However, it has been reported that several types of dermal melanocytosis could appear in adult life. We report here on an unusual case of acquired dermal melanocytosis occurring in a 51 year-old female. She had bilaterall brownish or slate-bluish pigmented patches on the face and the posterior auricular, supraclavicular, scapular and back areas. The skin biopsy specimen taken from the upper back revealed scattered, darkly pigmented, spindle-shaped cells and dendritic cells containing abundant golden brown pigment in the dermis.
Subject(s)
Adult , Female , Humans , Biopsy , Dendritic Cells , Dermis , Melanocytes , Mongolian Spot , Nevus , Nevus of Ota , Nevus, Blue , Parturition , SkinABSTRACT
We reported two cases of clinically typical melasma presenting with unusual histopathologic findings. In one case, the epidermal melanocytes were markedly increased in number and protruded into the dermis, and in the other case, increased epidermal pigmentation as well as dermal melanocytosis were found. We suggested that the various treatment modalities of melasma should be applied depend on its histopathologic finding.