Angiotropic melanocytes: Possibly a distinctive finding in extra-facial acquired dermal melanocytosis

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

Melanocitosis dérmica adquirida en la mano de un paciente pediátrico / Acquired dermal melanocytosis on the hand of a pediatric patient

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.

Asociación de melanocitosis dérmica con enfermedades lisosomales / Association of dermal melanocytosis with lysosomal storage diseases

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.

Acquired Dermal Melanocytosis of the Nose Successfully Treated with Neodymium-doped Yttrium Aluminum Garnet 1064 nm Laser / 대한피부과학회지

No abstract available.

Prevalence of blue spots among 1-10 years old children, Mongolia, 2014 / Монголын Анагаах Ухаан

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.

A Case of Hair Follicle Nevus with Dermal Melanocytosis / 대한피부과학회지

No abstract available.

Two Cases of Vitiligo Developed on the Persisting Dermal Melanocytosis: Is There a Difference between Epidermal Melanocytes and Dermal Melanocytes?

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.

Imatinib Mesylate Induced Acquired Dermal Melanocytosis / 대한피부과학회지

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.

An Unusual Case of Congenital Dermal Melanocytosis

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.

A Case of Uncommon Acquired Dermal Melanocytosis / 대한피부과학회지

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.

A Case of Uncommon Acquired Dermal Melanocytosis / 대한피부과학회지

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.

A Case of Acquired Dermal Melanocytosis Occurring on the Chest / 대한피부과학회지

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.

A Case of the Generalized Type of Acquired Dermal Melanocytosis with ABNOM and Acquired Bilateral Nevus of Ito-like Macules / 대한피부과학회지

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.

A Case of Dermal Melanocytosis with Features of Neurocristic Cutaneous Hamartoma / 대한피부과학회지

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.

Two Cases of Melasma with Unusual Histopathologic Findings

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.

A Case of Acquired Dermal Melanocytosis showing the Manifestations of Bilateral Variant of Nevus of Ito / 대한피부과학회지

There are a number of pigmentation disorders due to dermal melanocytes, including Mongolian spot, nevus of Ota, nevus of Ito and blue nevus, which usually appear at birth or in early childhood. But, several types of acquired dermal melanocytosis which usually appear in adult have been reported. A 67-year-old woman presented with asymptomatic, multiple, symmetric, bluish macules on the upper back for 10 years. Histopathologic findings showed some scattered spindle-shaped cells containing melanin pigment in the upper dermis and these cells were positive for S-100 protein stain. We suggest this case may be an acquired and bilateral variant of nevus of Ito considering the relationship between the acquired bilateral nevus of Ota-like macules and nevus of Ota.

Acquired Dermal Melanocytosis Occurring on the Hand

While most dermal melanocytoses are congenital or have an onset in early childhood, there is an acquired group with a late onset. We describe a case of aldolescent onset, unilateral, macular dermal melanocytosis on the hand. A 16-year-old girl presented with a 10-month history of gray-bluish pigmentation on the right palm and the volar side of the right fourth finger. The light microscopic studies showed spindle-shaped dermal melanocytes. The S-100 protein staining of these cells was positive and CD68 staining was negative. Dermal melanocytosis of the macular type, as observed in our case, is very rare.

Two Cases of Acquired Dermal Melanocytosis / 대한피부과학회지

Dermal melanocytosis is observed in various congenital conditions including nevus of Ota, nevus of Ito and Mongolian spot. It usually appears at birth or in early childhood. Several types of acquired dermal melanocytosis which usually appear in adult life have been reported. As the late onset has been stressed, the term acquired is used. We report two cases of acquired dermal melanocytosis on the forearm and the back without any similar pigmentation elsewhere on the body. The first case is a 47-year-old man who had numerous grey-blue colored macules and patches on the upper back. Histopathologic findings showed a large number of spindle-shaped cells containing melanin granules in the dermis. On electron microscopy, dermal melanocytes with stage 3, 4 melanosomes and lipid droplet were observed. The second one is a 13-year-old girl who had a 3.8 X 2.5 cm sized dark grey colored patch on the left forearm. Histopathologic and EM finding were similar to case 1. We treated both cases with topical cream(retinoid and hydroquinone), but could not observe improvement. Their lesions have persisted without any change.

A Case of Phakomatosis Pigmentovascularis with Eye Involvement / 대한피부과학회지

Phakomatosis pigmentova.scularis is a disease complex with dermal melanocyto sis such as nevus of Ota, nevus of Ito, or Mongolian spots and extensive hemangiomatous change such as nevus flammeus, which are associated with systemic organ involvement. We report a 23-year-old female, who showed bilateral nevus of Ota and nevus of Ito with widespread nevus flammeus and Mongolian spots since birth. She was also associated with megalocornea due to congenitol glaucoma and cataract.

Hairy Blue Nevus

A 2-month-old female infant presented with a bluish-black, flat, hairy patch, 1.2cm in diameter, on the posterior aspect of her neck since birth. Histologic sections from the lesion showed numerous diffusely scattered dermal melanocytes throughout the dermis. The histopathologic and clinical findings were not fully oonsistent with any other known dermal melanocytosis. The diagnosis of “hairy blue nevus” was proposed for this unique lesion.