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.

Schamberg's disease: case report with therapeutic success by using colchicine

Abstract: Pigmented purpuric dermatoses (PPD) include a spectrum of diseases with different clinical aspects, but with similar histopathological features. Specific clinical findings allow the division of PPD in variants. Schamberg's disease is the most common. Treatment is sometimes ineffective and recurrences are common. There are reports of patients who responded well to the use of colchicine. We report the case of a 32-year-old woman, previously healthy, with a history of onset of asymptomatic lesions in legs. She presented purpuric skin eruptions and brownish stains diffusely distributed in the lower limbs. Biopsy was compatible with PPD. We decided for the introduction of colchicine, with good clinical response. The patient has been followed on outpatient basis for ten months without recurrence.

A case of pigmented Bowen's disease

ABSTRACT Pigmented Bowen's disease is a rare subtype of Bowen's disease. Clinically it presents as a slow-growing, well-defined, hyperpigmented plaque, and should be included as a differential diagnosis of other pigmented lesions. The authors describe a challenging case of pigmented Bowen's disease with non-diagnostic dermscopy findings.

Cutaneous polyarteritis nodosa causing refractory skin deformation and pigmentation as sequel

Abstract: A 39-year-old woman presented with prominent and painful livedo reticularis lesions spreading on her upper and lower extremities. Histopathologically, the small-to medium-sized arteries in the deep dermis and subcutis showed necrotizing vasculitis with cellular infiltration, suggesting cutaneous polyarteritis nodosa. The serum levels of inflammatory markers normalized with aspirin 100mg/day and prednisolone 10mg/day within 2 months, and there was no other skin or organ involvement over 18 months of follow up. However, serious refractory skin depressions and pigmentation remained after two years of treatment. This suggests the importance of early and aggressive therapy for cutaneous polyarteritis nodosa to prevent unsightly skin sequel, as well as control of disease activity.

Dermatoscopy-guided therapy of pigmented basal cell carcinoma with imiquimod

Abstract BACKGROUND: Dermatoscopy is a non-invasive diagnostic tool used to examine skin lesions with an optical magnification. It has been suggested as a useful tool for monitoring therapeutic response in lentigo maligna patients treated with imiquimod. OBJECTIVE: To examine the accuracy of dermatoscopy as a tool to monitor the therapeutic response of pigmented basal cell carcinoma treated with imiquimod. METHOD: The authors designed a prospective study. Patients with pigmented basal cell carcinoma were included and data regarding the dermatoscopy features were collected following the Menzies criteria, prior to initiating the imiquimod treatment. Subsequent dermatoscopic evaluations were performed at weeks 4 and 8, following imiquimod discontinuation. RESULTS: Twenty lesions were included. The most common pigmented dermatoscopy features were large blue-grey ovoid nests (80%), followed by blue-grey globules (50%) and leaf-like areas (30%). No spoke wheel areas were observed. In 17 out of 20 patients, a response was noted during the first evaluation at 4 weeks, while the clearance was noted at the second check-up after 8 weeks. In two patients, the clearance was found at the initial evaluation at 4 weeks, while in one patient, the response remained unchanged. Blue-grey globules were the fastest to exhibit clearance (50% at week 4), followed by leaf-like areas (15%) and large blue-grey ovoid nests (6.25%). CONCLUSION: According to our results, dermatoscopic evaluation enhances the accuracy in the assessment of the clinical response to imiquimod in pigmented basal cell carcinoma.

Dermatoscopic findings of pigmented purpuric dermatosis

Abstract: Background: Pigmented purpuric dermatosis is a chronic skin disorder of unknown aetiology characterised by symmetrical petechial and pigmented macules, often confined to the lower limbs. The aetiology of pigmented purpuric dermatosis is unknown. Dermatoscopy is a non-invasive diagnostic technique that allows the visualisation of morphological features invisible to the naked eye; it combines a method that renders the corneal layer of the skin translucent with an optical system that magnifies the image projected onto the retina. Objectives: The aim of this study is to investigate the dermatoscopic findings of pigmented purpuric dermatosis. Methods: This study enrolled patients diagnosed histopathologically with pigmented purpuric dermatosis who had dermatoscopic records. We reviewed the dermatoscopic images of PPD patients who attended the outpatient clinic in the Istanbul Dermatovenereology Department at the Bezmialem Vakıf University Medical Faculty. Results: Dermatoscopy showed: coppery-red pigmentation (97%, n = 31) in the background, a brown network (34%, n = 11), linear vessels (22%, n = 7), round to oval red dots, globules, and patches (69%, n = 22; 75%, n = 24; 34%, n = 11; respectively), brown globules (26%, n = 8) and dots (53%, n = 17), linear brown lines (22%, n = 7), and follicular openings (13%, n = 4). Conclusion: To our knowledge, this is the first study to report the dermatoscopy of pigmented purpuric dermatosis. In our opinion, dermatoscopy can be useful in the diagnosis of pigmented purpuric dermatosis.

Dermatosis cenicienta: revisión y diagnóstico diferencial con otros trastornos pigmentarios idiopáticos / Ashy dermatosis: revision and differential diagnosis with other idiopathic pigmentary disorders

La dermatosis cenicienta es un trastorno pigmentario infrecuente que se presenta en individuos de piel morena, especialmente en centroamericanos. Las lesiones consisten en manchas hiperpigmentadas de coloración gris-azulada, principalmente localizadas en la cara, tronco y brazos. Su principal diagnóstico diferencial debe ser planteado con tres dermatosis pigmentarias de origen idiopático: eritema discrómico perstans, pigmentación macular eruptiva idiopática y liquen plano pigmentoso. Hay consenso en considerar al eritema discrómico perstans como la misma enfermedad, pero con un borde eritematoso transitorio. La pigmentación macular eruptiva idiopática es clínicamente similar, pero sin una dermatitis de interfase al estudio histopatológico, y sólo muestra una pigmentación de la capa basal, incontinencia pigmentaria y melanófagos en la dermis. Finalmente, el liquen plano pigmentoso debe ser considerado como una variedad pigmentada de liquen plano.

Ashy dermatosis is an infrequent disorder of pigmentation, which predominantly occurs in darkly pigmented individuals, specially in central americans. The lesions consist of bluish-gray hyperpigmented patches mainly located on the face, trunk and arms. Its main differential diagnosis must be raised with three idiopathic pigmentary disorders: erythema dyschromicum perstans, idiopathic eruptive macular pigmentation and lichen planus pigmentosus. It has been widely accepted that erythema dischromicum perstans is the same disease, but with a transient erythematous border. Idiopathic eruptive macular pigmentation is clinically similar but without an interfase dermatitis at histopathologic studies, only showing increased pigmentation of the basal layer and pigmentary incontinence and many melanophages in the dermis. Finally, lichen planus pigmentosus must be considered as a pigmented variant of lichen planus.

Dermatosis purpúrica pigmentaria, variante granulomatosa, a propósito de un caso / Granulomatous variant of chronic pigmented purpuric dermatosis: case report

La dermatosis purpúrica pigmentaria (DPP) corresponde a un grupo de enfermedades caracterizadas clínicamente por petequias y coloración bronce de la piel. Generalmente se localiza en las extremidades inferiores y se describen varios tipos, dentro de los cuales se encuentra la variante granulomatosa. Se trata de una dermatopatía benigna, poco frecuente, de etiología aún no precisada y de difícil manejo. Se presenta el caso de una paciente de 48 años, sexo femenino, sin antecedentes mórbidos, que es derivada a dermatología desde reumatología, con el diagnóstico clínico de vasculitis, por la aparición de lesiones maculares y papulares eritematovioláceas, confluentes y pruriginosas en extremidades. Presenta todos sus exámenes normales. Se planteó, entonces, una acroangiodermatitis o liquen plano. Sin embargo, fue necesario llegar al estudio histopatológico para llegar al diagnóstico de dermatosis purpúrica pigmentaria, variante granulomatosa. Si bien se considera que la DPP granulomatosa es una entidad poco frecuente, la literatura sugiere que es una entidad subdiagnosticada. Se debe plantear como diagnóstico diferencial de lesiones cutáneas en extremidades inferiores, lo que determina la importancia de realizar el estudio histopatológico. De esta forma, la DPP granulomatosa será cada vez más común y se plantearán alternativas de tratamiento mejores a las actuales.

The pigmented purpuric dermatoses are a group of diseases characterized clinically by petechiae and bronze skin color, usually affecting the lower extremities. Several types are described and one of them is the granulomatous variant. This is a still rare and benign skin disease of unknown etiology. We present the case of a 48 years old female, previously healthy, derived from rheumatology to dermatology, with the clinical diagnosis of vasculitis due to the presence of confluent erythematous macular and papular lesions, mildly pruritic in lower extremities. Her tests were all in normal ranges. Therefore, an acroangiodermatitis or lichen planus were considered. However, it was necessary to perform a histopathology study to reach the final diagnosis of granulomatous pigmented purpuric dermatosis. It is considered that Granulomatous DPP is a rare entity, but the literature suggests that it is underdiagnosed. It should always be considered as a differential diagnosis of cutaneous lesions located in lower extremities, which determines the importance of histopathology. Thus, the granulomatous DPP will become more common and will be posible to propose better treatment alternatives.

Phototherapy as an effective treatment for Majocchi's disease - Case report

Pigmented purpuric dermatoses are chronic and relapsing disorders characterized by a symmetrical rash of petechial and pigmentary macules, mainly confined to the lower limbs. Purpura annularis telangiectodes of Majocchi is a less common variant of Pigmented purpuric dermatoses characterized by punctate telangiectatic macules progressing to annular, hyperpigmented patches with central clearing and infrequent atrophy. A 12 year-old girl presented with asymptomatic round to oval reddish brown macules, present symmetrically over her lower and upper limbs for 3 years. Few lesions were annular in shape. Biopsy from the lesion was compatible with Pigmented purpuric dermatoses. On the basis of clinical and histopathological findings, a diagnosis of Purpura annularis telangiectodes of Majocchi was made. The patient began phototherapy thrice a week and showed excellent response.

Eritema discromicum perstans (EDP) o dermatosis cenicienta: revisión a propósito de dos casos / Erythema discromicum perstans (EDP) or cystic dermatosis: review of two cases

El eritema discromicum perstans (EDP) o Dermatosis cenicienta es una entidad clínica poco común, de curso crónico e irreversible. Se presenta como máculas hiperpigmentadas color gris-ceniza, de tamaño y forma variable, distribuidas en cara, tronco, y extremidades. En Chile existen pocos casos reportados. La etiología es actualmente desconocida. Los hallazgos histopatológicos no son específicos de EDP, por lo que el diagnóstico es clínico e histopatológico. Aunque se han reportado múltiples opciones de tratamiento, ninguno ha demostrado ser efectivo de manera consistente. Presentamos dos casos de EDP y una revisión de los principales aspectos clínicos, epidemiológicos e histopatológicos de esta entidad.

Erythema dyschromicum perstans (EDP) or cinderella Dermatosis is an uncommon clinical entity, of chronic and irreversible course. Clinically it appears as hyper pigmented gray-ashy macules, of variable size and shape, distributed on face, trunk, and extremities. There are few cases reported on Chile. Etiology is currently unknown. The histopathological features are not characteristic of EDP, so the diagnosis is based upon clinical findings plus histopathology. Although there are several treatment options, none of them have been demonstrated to be consistently effective. We present two cases of EDP and a review of the clinical, epidemiological, and histopathological features of this entity.

Acropigmentación reticulada de Dohi: revisión de genodermatosis con pigmentación reticular a partir de un caso clínico / Reticulated acropigmentation of Dohi: review of genodermatosis with reticular pigmentation

La acropigmentación reticulada de Dohi (ARD) o discromía simétrica hereditaria se encuentra dentro de los desórdenes con pigmentación reticular hereditaria. Es una rara entidad que se describió por primera vez en 1929 en Japón por Toyama. Presenta herencia autosómica dominante y el gen responsable se encuentra en el cromosoma 1q21 que codifica para una proteína ARN-adenosina desaminasa (ADAR1 o DSRAD). A pesar, que es un desorden benigno y no reviste complicaciones suele ser estéticamente desfigurante. A continuación, se presenta un caso clínico y se realizará una revisión de la literatura de los otras genodermatosis con pigmentación reticular.

Hereditary symmetric dyschromatosis, also known as reticulated acropigmentation of Dohi is included within a spectrum of reticulate pigment disorders of the skin. It’s a pigmentary disorder characterized by the presence of hypopigmented and hyperpigmented macules giving a reticular pattern in acral areas. It is a rare entity first described in 1929 by Toyama in Japan. It has autosomal dominant inheritance and the responsible gene codes for an RNA adenosine deaminase protein (ADAR1 or dsRAD) on chromosome 1q21. Although, it`s a benign disorder, it can be aesthetically disfiguring. We present a case report and review of the literature of genodermatosis with reticular pigmentation.

Pigmented Bowen's disease

Pigmented Bowen's disease is rare, though more prevalent in men. It presents as a well-delineated plaque in areas unexposed to sun. There are reports of association with seborrheic keratosis, solar lentigo or exuberant pigmentation of genital and intertriginous regions. A specific dermoscopy finding is the presence of brown or gray dots in regular arrangement and coiled or dotted vessels. Thus, we aim to raise awareness of the diagnosis of pigmented Bowen's disease in pigmented lesions.

Poikilodermatous changes on the forearms of a woman practicing aroma-therapy: extracervical poikiloderma of Civatte?

We report the case of a 48-year-old, Caucasian female who presented with slowly progressing asymptomatic poikilodermatous changes of the extensor aspects of the forearms. She also had typical Poikiloderma of Civatte on the V of the neck and erythemato-telangiectatic rosacea of the central face. The patient had been practicing aroma-therapy for many years. Histologic examination revealed findings consistent with PC. Patch-testing revealed positive reactions to Fragrance mix and Nickel sulphate. Based on clinical and histological findings, a diagnosis of extracervical PC was suggested. PC with extra-cervical or extra-facial involvement is rare. In addition, this case supports the theory that contact sensitization to fragrances may contribute to the development of PC.

Hallazgos dermatoscópicos en las dermatosis purpúras pigmentadas / Dermoscopy of pigmented purpuric dermatosis

Las Dermatosis Purpúricas Pigmentadas (DPP) son un grupo de desórdenes concaracterísticas clínico-histológicas similares y etiología desconocida. El patrón clínico consiste en lesiones purpúricas sobre una base hiperpigmentada, distribuidasgeneralmente de forma simétrica en miembros inferiores. En la mayoría de los casos su diagnóstico clínico es simple, aunque se confirma conla histología. Presenta una amplia lista de diagnósticos diferenciales que debemos descartar ante la aparición de lesiones sospechosas. Realizamos el estudio clínico, dermatoscópico e histopatológico de diez casos deDPP, encontramos en todos un patrón dermatoscópico característico denominado patrón purpúrico moteado, compuesto por puntos, glóbulos o parches rojos, que se encuentra sobre una pigmentación difusa marrón cobriza. Consideramos que estos hallazgos son útiles para confirmar la sospecha clínica...

Congenital agminated melanocytic nevus - case report / Nevo melanocitico congenito agminado - relato de caso

Agminated nevus is a cluster group of melanocytic nevi confined to a localized area of the body. There are many pigmented lesions described in the literature as agminated, such as blue nevi, multiple lentigines and Spitz nevi, but only a few cases of congenital agminated melanocytic nevi have been described. We report a case of a male child who presented with congenital agminated nevi, emphasizing the importance of physical examination, dermoscopy, histopathological evaluation, differential diagnosis and follow up to rule out the possibility of dysplastic or malignant changes.

Nevo agminado é um conjunto de lesões melanocíticas confinadas a uma área do corpo. Existem muitas lesões descritas na literatura como agminada s, como nevos azuis, lentigos múltiplos, nevo de Spitz, mas existem poucos casos de nevos melanocíticos agminados congênitos descritos. Relatamos o caso de um paciente do sexo masculino que se apresentou com um nevo agminado congênito, enfatizando a importância do exame físico, dermatoscopia, avaliação histológica, diagnóstico diferencial e seguimento para descartar a possibilidade de alterações displásicas ou malignas.

Cutaneous mosaicisms: concepts, patterns and classifications / Mosaicismos cutaneos: conceitos, padroes e classificacoes

A mosaic is an organism composed of two or more genetically distinct cell populations derived from a genetically homogeneous zygote. Cutaneous mosaicisms are the clinical expressions of these disorders. The main event which allows the existence of mosaicism is a genetic mutation, either structural or functional. Cutaneous mosaicisms usually manifest by specific patterns on the skin and the archetypic pattern is the system of Blaschko lines, but others include checkerboard, phylloid, large patches without midline separation and lateralization. Since 1901, when Blaschko lines were first described, the study of mosasicism has helped to elucidate the behavior of numerous genetic diseases, generating therapeutic perspectives for these pathologies, including the promising gene therapy.

Um mosaico é um organismo formado por duas ou mais populações de células geneticamente distintas originadas a partir de um mesmo zigoto geneticamente homogêneo. Os mosaicismos são as expressões clínicas dessa desordem, e a mutação gênica seu evento determinante, que pode ser tanto estrutural quanto funcional. Os mosaicismos cutâneos costumam se expressar em padrões específicos, dentre os quais podem ser mencionados as prevalentes linhas de Blaschko, o padrão "checkerboard", o padrão filóide, o padrão em placa sem separação na linha média e o padrão de lateralização, que serão abordados neste artigo. Desde 1901, momento da primeira descrição das linhas de Blaschko, o estudo dos mosaicismos tem contribuído para a elucidação do comportamento de numerosas desordens genéticas, de forma a criar perspectivas terapêuticas para essas doenças, incluindo a promissora terapia gênica.

Follicular red dots: a normal trichoscopy feature in patients with pigmentary disorders? / Pontos vermelhos foliculares: um achado tricoscópico normal em pacientes com distúrbios da pigmentação?

Follicular red dots have been described as a trichoscopic feature of active discoid lupus erythematosus of the scalp and its presence associated with a better prognosis. We report five patients with pigmentary disorders in whom follicular red dots were detected during scalp examination. We suggest that this pattern is probably related to the rich vasculature that naturally envelops the normal hair follicle. The possible implications of such proposition in cases of discoid lupus erythematosus and other scalp disorders are also discussed.

Pontos vermelhos foliculares foram descritos como achado tricoscópico de lupus eritematoso discóide do couro cabeludo em atividade e a presença destes associado a melhor prognóstico. Relatamos cinco pacientes com distúrbios da pigmentação nos quais pontos vermelhos foliculares foram detectados ao exame do couro cabeludo. Sugerimos que este padrão está provavelmente relacionado à rica vascularização que naturalmente envolve o folículo piloso normal. As possíveis implicações desta suposição nos casos de lupus eritematoso discóide e outras doenças do couro cabeludo também são discutidas.

Techniques of digital image analysis for histological quantification of melanin / Técnicas de análise de imagem digital para quantificação histológica da melanina

Morphometric analysis of tissue melanin may quantitatively contribute to research on pigmentation disorders. The authors present three methods for image analysis, which allow for identification of melanin-equivalent pixels in the epidermis using Fontana-Masson stain and, therefore, for the calculation of its percentage in the different epidermal layers. Moreover, they discuss the main elements related to the analysis and the need for rigorous standardization of the process.

A análise morfométrica da melanina tecidual pode subsidiar quantitativamente a pesquisa em discromias. Os autores demonstram três técnicas de análise de imagem digital que permitem a identificação dos pixels equivalentes à melanina na epiderme pela coloração de Fontana-Masson, possibilitando o cálculo da sua porcentagem nas diferentes camadas da epiderme, e discutem os principais elementos relacionados à análise e a necessidade de rigorosa padronização do processo.