Cutaneous hypopigmented lymphoproliferative disorders: a clinicopathological study of 41 cases / 中华皮肤科杂志

Objective:To investigate clinicopathological features of hypopigmented mycosis fungoides (HMF) and hypopigmented interface T-cell dyscrasia (HITCD) .Methods:A total of 41 patients with cutaneous hypopigmented lymphoproliferative diseases, who had complete clinicopathological data, were collected from Department of Dermatology, the Third People′s Hospital of Hangzhou from January 2015 to September 2020, and the clinicopathological and immunophenotypic features were analyzed. Comparisons of normally distributed measurement data were carried out using t test, comparisons of categorical data using Chi-square test or Fisher′s exact test, and comparisons of ranked data between 2 groups using rank-sum test. Results:All of the 41 patients clinically presented with irregular hypopigmentation, some of which was accompanied by erythema or furfuraceous scales. In terms of pathological features, 21 patients showed infiltration and aggregation of atypical lymphoid cells in the epidermis, which was consistent with typical pathological features of mycosis fungoides, and they were diagnosed with HMF; 20 patients showed vacuolar degeneration of the basal layer, accompanied by infiltration of lymphoid cells and mild epidermotropism, and they were diagnosed with HITCD. All immune cells expressed T-cell phenotype, and epidermal lymphocytes expressed a CD8-dominated phenotype in 14 (67%) cases of HMF and 13 (65%) of HITCD. In the epidermis, the total number of lymphocytes was significantly higher in the HMF group than in the HITCD group ( t= 1.81, P= 0.012) ; in the dermis, the number of CD4 + lymphocytes and CD8 + lymphocytes, and the total number of lymphocytes were all significantly higher in the HMF group than in the HITCD group ( t= 2.64, 1.51, 2.60, P= 0.012, 0.002, 0.001, respectively) . All patients were treated with narrow-band ultraviolet B radiation. Among 34 patients who completed the follow-up, 30 achieved complete clearance of skin lesions without recurrence, including all patients with HITCD, and 4 with HMF achieved partial regression of the lesions. Conclusions:Compared with HMF, HITCD presents different pathological characteristics and benign biological behaviors. Thus, HITCD should be distinguished from HMF as an independent disease. Phototherapy alone is effective for the treatment of HITCD.

In vitro culture of melanocytes from segmental vitiligo-like nevus depigmentosus lesions and its clinical significance / 中华皮肤科杂志

Objective:To assess the value of culture of epidermal melanocytes from negative-pressure suction blisters in the auxiliary diagnosis of segmental vitiligo-like nevus depigmentosus.Methods:Between June 2019 and March 2020, 8 patients with segmental vitiligo-like nevus depigmentosus, who met the Coupe′s clinical diagnostic criteria, were enrolled from Department of Dermatology, Hangzhou Third People′s Hospital. All patients were evaluated by the Wood′s lamp, reflectance confocal microscopy (RCM) , 308-nm excimer laser radiation, and in vitro culture of epidermal melanocytes from negative-pressure suction blisters. Results:Among the 8 patients, fluorescence was observed in 6 under the Wood′s lamp, dermal papillary rings were incomplete or absent in 4 as shown by RCM, and 5 experienced no repigmentation after 308-nm excimer laser radiation. Among the 8 patients, in vitro cultured lesional melanocytes were all positive for ferrous sulfate staining, yellowish-white precipitates were obtained after digestion and centrifugation of the melanocytes, and stage Ⅰ-Ⅲ melanosomes were observed in the cytoplasm of melanocytes under the electron microscope; however, the precipitates were black in color after digestion and centrifugation of the melanocytes collected from the normal skin tissues at the contralateral anatomical site, and stageⅠ-Ⅳ melanosomes were seen in the cytoplasm of the melanocytes under the electron microscope. Conclusion:Culture of epidermal melanocytes from negative-pressure suction blisters may facilitate the diagnosis of segmental vitiligo-like nevus depigmentosus.

Ensaio clínico cego randomizado comparando MMP®, MMP® com 5-FU e injeção intradérmica de 5-FU para o tratamento da hipomelanose gutata idiopática: um estudo- piloto / A single blind, randomized clinical trial comparing MMP®, MMP® with 5-FU, and 5-FU intradermal injection for the treatment of idiopathic guttate hypomelanosis: a pilot study

Introdução: A hipomelanose gutata idiopática (IGH) é uma manifestação comum de fotoenvelhecimento, ainda sem tratamento padrão, apresentando resultados variados às intervenções. Atualmente, no Brasil, o uso de microagulhamento associado ao 5-fluorouracil (5-FU) tem sido proposto para o tratamento da IGH. Objetivo: Comparar três tratamentos, quais sejam: o uso do MMP® (microinfusão de medicamentos na pele) com 5-FU, MMP® apenas para microagulhamento, e com o 5-FU intralesional injetado com seringa de insulina no tratamento da IGH. Métodos: Em um ensaio clínico randomizado e cego, comparamos o MMP® ao 5-FU com: microagulhamento isolado e com 5-FU intralesional por punção para o tratamento de 180 lesões de IGH no antebraço de nove pacientes. Resultados: Após duas sessões de tratamento, o 5-FU intralesional foi o tratamento mais efetivo, com significância estatística quando comparado ao uso de microagulhamento. A eficácia da MMP + 5-FU foi inferior a 5-FU injetável e superior ao microagulhamento isoladamente, embora sem significância estatística. Conclusões: A aplicação intralesional do 5-FU foi mais eficaz no tratamento da leucodermia solar. O uso de menor quantidade de medicamentos é a grande vantagem da técnica MMP + 5-FU. São necessários mais estudos para padronizar estas técnicas.

Introduction: Idiopathic guttate hypomelanosis (IGH) is a common photoaging manifestation, with no standard treatment and presenting mixed results to interventions. In Brazil, the use of micro-needling associated with 5-fluorouracil (5-FU) has been proposed to treat IGH. Objective: To compare the use of MMP® (micro-infusion of drugs on the skin) with 5-FU, with MMP® only as micro-needling (with no drugs), and intralesional 5-FU injected with an insulin syringe in the treatment of IGH. Methods: In a single blind randomized clinical trial, we compared the three treatments: MMP® versus MMP with 5-FU and intralesional 5-FU injection for 180 IGH lesions in the forearm of nine patients. Results: After two treatment sessions, 5-FU alone was the most effective treatment, with statistical significance, compared with micro-needling alone. MMP+5-FU efficacy was lower than intralesional 5-FU injection and higher than micro-needling alone, although without statistical significance. Conclusions: The intralesional application of 5-FU was more effective in the treatment of solar leukoderma. The use of a smaller quantity of medication is the great advantage of the MMP + 5-FU technique. Further studies are needed to standardize these techniques.

Enxerto dermoepidérmico obtido por remoção da pápula formada pós-enxertia com punch em vitiligo: melhora do padrão em paralelepípedo / Dermoepidermal grafting obtained by shave excision of papule formed post punch grafting in vitiligo: improvement of the cobblestone pattern

O vitiligo é uma discromia adquirida que afeta 0,1-3% da população mundial e pode causar sofrimento psicossocial significativo nos pacientes. O manejo cirúrgico tornou-se importante nos últimos anos. O minienxerto é uma das abordagens mais baratas e fáceis entre todos os tratamentos cirúrgicos disponíveis. Uma limitação desta técnica é a possibilidade de repigmentação em forma de paralelepípedos. Descreve-se uma nova técnica com enxerto dermoepidérmico obtido por remoção da pápula formada pós-enxertia com punchs no vitiligo. Essa técnica pode melhorar o padrão em paralelepípedos no local doador e a repigmentação em ambas as áreas, doadora e receptora.

Vitiligo is an acquired dyschromia that affects 0,1-3% of the world population and can cause significant psychosocial distress in patients. Surgical management became essential in the last years. Mini-punch grafting is one of the cheapest and most straightforward approaches to all surgical treatments available. A limitation of this technique is the possibility of cobblestone-like repigmentation. We describe a new technique with a dermoepidermal grafting obtained by shave excision of papule formed post punch grafting in vitiligo. It could improve the cobblestone pattern in the donor site and the repigmentation in both donor and recipient areas

Clinical analysis of vulvar lichen simplex chronicus / 中华皮肤科杂志

Objective To investigate clinical features of vulvar lichen simplex chronicus (VLSC).Methods Clinical data were collected from 137 VLSC patients who visited a Vulvar Clinic in the Department of Dermatology,Beijing Hospital from 2017 to 2018,and analyzed retrospectively.Non-normally distributed measurement data (age and disease duration) were described as median (P25,P75),and analyzed by using rank sum test,and enumeration data were compared by using chi-square test.Results Among the 137 patients with VLSC,the age at onset was 32.0 (25.5,40.0) years,and the disease duration was 36.0 (15.0,72.0) months.Thirty-two (23.4％) patients had a history of atopic diseases,and the age at onset of VLSC was significantly lower in these patients with a history of atopic diseases (29.5 [25.0,35.8]years) than in those without a history of atopic diseases (33.0 [27.0,41.0] years,Z =2.03,P =0.042).The most frequently involved site was the labia majora (130/137,94.9％),and the labia minora was rarely involved (13/137,9.5％).Bilateral lesions were observed in 103 (75.2％) patients,and hypopigmentation occurred in 8 (5.8％) patients.All the patients experienced itching to different extents,including moderate itching in 44 (32.1％) cases and severe itching in 80 (58.4％).The ratio of patients with severe itching to those with disease duration ＞ 2 years (68.1％) was significantly higher than that of patients with severe itching to those with disease duration ＜ 2 years (47.7％,x2 =5.830,P =0.016).Patients reported that local wetness and sweating (55 cases,40.1％),spicy diet (41 cases,29.9％) and mental stress (36 cases,26.3％) could aggravate itching,Conclusions VLSC commonly occurs in patients aged 20-39 years,and atopic predisposition may be an important factor for VLSC.VLSC mostly involves bilateral labia majora,and the longer the disease duration,the more severe the itching.

First report of complete albinism in Mazama americana (Erxleben, 1777) in the Biological Reserve of Tapirapé, Oriental Amazon, Brazil

Albinism is a genetic condition that results in total hypopigmentation of the eyes, fur, skin, hair, scales, and feathers of an organism. Albinism might result in a selective disadvantage for affected animals. Cases of albinism have been previously recorded in Neotropical vertebrates, such as reptiles, mammals, birds, and fish. However, observing albinism in a wild population is still considered to be a rare event. This paper reports a unique case of complete albinism in a red-brocket deer (Mazama americana) living in the Brazilian Amazon rainforest. The individual was observed within the Biological Reserve of Pará State, one of the most deforested regions of the Brazilian Amazon. The survival of the albino red-brocket deer in the wild can be related to mechanisms of apostatic selection, which theorize the survival of individual prey animals whose mutations make them less likely to be attacked by predators. In other words, the more different a prey animal is from others, the less likely it will be targeted by predators. The high abundance prey animals within the Biological Reserve of Tapirapé seems to support this prediction. This report exemplifies the importance of monitoring the biodiversity and promoting the conservation of favorable habitats to support species multiplicity in highly fragmented regions, as in the Brazilian Amazon.

Depigmentation in a Patient with Chronic Myeloid Leukemia during Chemotherapy with Dasatinib / 대한피부과학회지

No abstract available.

Frequency of leucism in a colony of Anoura geoffroyi (Chiroptera: Phyllostomidae) roosting in a ferruginous cave in Brazil / Frequência de leucismo em uma colônia de Anoura geoffroyi (Chiroptera: Phyllostomidae) abrigada em uma caverna ferruginosa no Brasil

Abstract: We provide the first report of the frequency of leucism for a species of Neotropical bat. Leucism is an anomaly of the skin pigmentation of an animal that manifests itself as the total or partial loss of the natural color of the species, and can affect part of or the entire body. During a study involving capture and marking individuals of a population of A. geoffroyi residing in a cave in the Brazilian state of Minas Gerais, seven individuals with some degree of depigmentation were recorded out of 616 individuals marked, for a frequency of 1.1%. Since leucism is due to recessive gene expression, these findings may indicate that the population is isolated and possesses a high level of endogamy. Factors that may be responsible for this condition in the studied population remain unknown.

Resumo: Relatamos aqui o primeiro registro de frequência de leucismo em uma espécie de morcego Neotropical. Leucismo é uma anomalia na pigmentação da pele dos animais que consiste na perda total ou parcial da cor natural da espécie, podendo acometer partes ou todo o corpo do animal. Em um estudo de captura e marcação de indivíduos de uma população de A. geoffroyi abrigada em uma caverna localizada no estado de Minas Gerais, foram registrados sete indivíduos com algum grau de despigmentação dentre 616 indivíduos marcados, representando 1.1% da população. Leucismo é um tipo de anomalia de coloração de expressão gênica recessiva, portanto, esse resultado pode indicar isolamento da população e nível elevado de endogamia. Fatores que podem levar à esta condição nessa população são desconhecidos.

Occurrence of tayras (Eira barbara Linnaeus, 1758) with anomalous coloration in Cerrado remnants in the state of São Paulo, Brazil / Ocorrência de iraras (Eira barbara Linnaeus, 1758) com coloração anômala em remanescentes de Cerrado no estado de São Paulo, Brasil

Abstract: Coloration anomalies are mainly genetically-based disorders in which body pigmentation is either reduced (hypopigmentation) or produced in excess (melanism), in parts or the totality of the body. Cases of hypopigmentation have been documented in many neotropical mammals, including the tayra (Eira barbara Linnaeus, 1758). We expand the account of anomalous coloration occurrence presenting new registers of hypopigmented tayras in Brazil. Data was collected during a mammal survey carried out in three agricultural landscapes within the Cerrado domain in the northeast of the state of São Paulo. We obtained two kinds of records of hypopigmented tayras, one from direct sighting and the other from a camera-trap. We discuss the likely implications of this conspicuous coloration to tayras and highlight some possibilities of study.

Resumo: Colorações anômalas são desordens geralmente de origem genética, em que a pigmentação do corpo é reduzida (hipopigmentação) ou excessiva (melanismo), em partes ou totalmente. Casos de hipopigmentação já foram documentados em muitos mamíferos neotropicais, incluindo a irara (Eira barbara Linnaeus, 1758). Nós expandimos os relatos de ocorrência de colorações anômalas apresentando novos registros de iraras hipopigmentadas no Brasil. Dados foram coletados durante um levantamento de mamíferos em três paisagens agrícolas no domínio do Cerrado no nordeste do estado de São Paulo. Obtivemos dois tipos de registros de iraras hipopigmentadas, sendo um por avistamento e outro por armadilhas fotográficas. Nós discutimos possíveis implicações ecológicas dessa coloração em iraras e sugerimos novas possibilidades de estudos.

Patrón cutáneo pigmentario de disposición lineal en un niño, vitíligo segmentario vs. hipomelanosis de Ito / Linear disposition pigmentary skin pattern in a child, segmental vitiligo vs. Hypomelanosis of Ito

Introducción: algunas enfermedades dermatológicas siguen disposición con patrones lineales. Con hipopigmentación en la infancia se encuentran el vitíligo segmentario, que sigue los dermatomas, aunque puede seguir las líneas de Blaschko y la hipomelanosis de Ito, que a su vez sigue las líneas de Blaschko. Estas dermatosis son infrecuentes en la práctica dermatólogica. Objetivo: profundizar en los elementos diagnósticos que permiten diferenciar dos dermatosis clínicamente caracterizadas por hipopigmentación segmentaria lineal de tipo blaschkoide y el tratamiento. Presentación del caso: a la consulta de Genodermatosis en Las Tunas acude un niño con máculas acrómicas en hemicuerpo izquierdo, sin otras alteraciones. Después de ser evaluado por varias especialidades (Dermatología, Genética, Pediatría, Oftalmología y Neurología), se determina que solo presentaba afectación cutánea, se le realizó biopsia de piel, que corroboró el diagnóstico de vitíligo segmentario. Conclusiones: se presenta el caso porque el vitíligo segmentario es infrecuente, sigue un patrón lineal que puede ser diferenciado de otra dermatosis infrecuente, como la hipomelanosis de Ito, y en el tratamiento es importante brindar apoyo psicológico al paciente para favorecer la obtención de mejores resultados con la Melagenina Plus(AU)

Introduction: some dermatological diseases are still available with linear patterns. In childhood with hypopigmentation can be found segmental vitiligo (which follows the dermatomes although it can follow the lines of Blaschko), and Hypomelanosis of Ito (which in turn follows the lines of Blaschko). These dermatoses are infrequent in dermatological practice. Objective: to deepen into the diagnostic elements that allows the differentiation of two dermatoses clinically characterized by linear segmental hypopigmentation of blaschkoid type and treatment. Case presentation: a child attends to the consultation of Genodermatoses in Las Tunas presenting acromic macules in left half of the body, without other alterations. After being evaluated by several specialties (Dermatology, Genetics, Pediatrics, Ophthalmology and Neurology), it was determined that only skin affectation was present. A skin biopsy was performed, which corroborated the diagnosis of segmental vitiligo. Conclusions: The case is presented because segmental vitiligo is infrequent, it follows a linear pattern that can be differentiated from another uncommon dermatosis, such as Hypomelanosis of Ito, and in the treatment it is important to provide psychological support to the patient to favor obtaining better results with Melagenina Plus(AU)

Artemisia capillaris Thunb. inhibits melanin synthesis activity via ERK-dependent MITF pathway in B16/F10 melanoma cells / 대한수의학회지

Genus Artemisia occurs as a hardy plant and has a wide range of culinary and medicinal features. In this study, we aimed to describe the melanin inhibitory activity of one Artemisia species, i.e., Artemisia capillaris Thunb. Ethanol extracts of fermented Artemisia capillaris (Art.EtOH.FT) and non-fermented Artemisia capillaris (Art.EtOH.CT) were tested for their ability to inhibit tyrosinase activity and melanin pigmentation. Both extracts showed dose-dependent inhibition against α-melanocyte stimulating hormone-stimulated melanin formation and tyrosinase activity, without cytotoxicity. At 100 µg/mL, both extracts showed greater inhibition than kojic acid, the positive control. Protein expressions of microphthalmia-associated transcription factor (MITF), tyrosinase (TYR), tyrosinase-related protein 1 (TRP-1), and tyrosinase-related protein 2 (TRP-2) at the transcriptional level were determined by using real-time and semi-quantitative polymerase chain reaction. To complete the mechanistic study, presences of upstream elements of MITF, the phosphorylated-extracellular signal-regulated kinase (p-ERK), and phosphorylated-mitogen-activated protein kinase kinase (p-MEK) were confirmed by using western blot analysis. Expressions of p-TYR, p-TRP-1 and p-TRP-2, downstream factors for p-ERK and p-MITF, were translationally inhibited by both extracts. Art.EtOH.FT induced more potent effects than Art.EtOH.CT, especially signal transduction effects. In summary, Artemisia capillaris extracts appear to act as potent hypopigmentation agents.

Familial progressive hyper- and hypopigmentation: a report on a Chinese family and evidence for genetic heterogeneity

Abstract Background: Familial progressive hyper- and hypopigmentation (FPHH) is a rare genodermatosis that is characterized by diffuse hyper- and hypopigmented spots on the skin and mucous membranes. It is caused by a pathogenic mutation of the KITLG gene. Objectives: To investigate the clinical features and mutation of the KITLG gene in a Chinese family with FPHH. Methods: Histopathological and immunohistochemical analysis of lesions from the proband was performed. The KITLG gene was screened for the presence of mutations. Results: A Chinese family containing 14 individuals with FPHH was described, and the proband was a 5-year-old girl showing diffuse hyper- and hypopigmented lesions on her extremities and trunk. Histopathological and immunohistochemical staining for S100 and HMB45 of skin biopsy specimens from the hyperpigmented areas showed a striking increase in melanin throughout the epidermis, especially in the basal cell layer, and staining of hypopigmented area specimens displayed lower levels of melanin in the epidermis. Mutation analysis of the KITLG gene was performed, but no mutation was found. Study limitations: The new pathogenic gene was not found. Conclusion: A family with FPHH was described. Analysis revealed that its members did not have any mutations of the KITLG gene, which provided evidence for genetic heterogeneity of this genodermatosis.

Treatment of Laser Therapy-Induced Punctate Leukoderma Using a 308-nm Excimer Laser

Punctate leukoderma presents as numerous, distinct, round or oval depigmented spots. Recently, laser therapy-induced punctate leukoderma associated with various Q-switched laser and carbon dioxide laser have been reported. A 25-year-old man presented with numerous, discrete, round, confetti-like, depigmented macules on his left neck. He had undergone 3 sessions of 532-nm Q-switched Neodymium: Yttrium-Aluminum-Garnet laser treatment for café-au-lait macules three years ago. After the last laser treatment session, the punctate leukoderma had been developed. We started treatment with the 308-nm excimer laser twice a week. After 7 months of treatment duration, complete repigmentation was achieved without serious adverse effects. We recommend the 308-nm excimer laser as an effective treatment modality for laser therapy-induced punctate leukoderma.

Reflectance confocal microscopy characteristics of childhood hypopigmented lichen striatus / 中华皮肤科杂志

Objective To investigate reflectance confocal microscopy (RCM) characteristics of childhood hypopigmented lichen striatus.Methods RCM was performed to image skin lesions and perilesional normal skin in 11 children with hypopigmented lichen striatus.Then,confocal microscopic findings of the skin lesions were compared with pathological findings.Results Histopathological examination of the skin lesions showed mild intercellular and intracellular edema,thickening of prickle cell layer to different extent,local liquefaction degeneration of basal cells,and perivascular infiltration of plenty of lymphocytes and a few melanophages in the superficial dermis.RCM horizontal images showed multifocal liquefaction degeneration of basal cells,which caused indistinct dermo-epidermal junction,incomplete or unclear dermal papillary rings,infiltration of many highly refractive melanophages and slightly to moderately refractive inflammatory cells in papillary and superficial dermis.Conclusion RCM characteristics can serve as strong evidences for the diagnosis and differential diagnosis of childhood hypopigmented lichen striatus.

Do you know this syndrome? Dyspigmentation along the Blaschko lines caused by trisomy 7 mosaicism

Abstract Dyspigmentation along the Blaschko lines is strongly suggestive of a mosaic skin disorder. We report a 9-year-old male patient who presented with swirls and streaks of both hypo and hyperpigmentation involving the entire body. Additionally, he had hypertrichosis, musculoskeletal and minor neurodevelopment abnormalities but no intellectual disability. Cultured fibroblast displayed trisomy 7 mosaicism, which can explain this pigmentary phenotype. Widespread dyspigmentation associated with involvement of other organs should prompt systemic examination to detect additional anomalies and genetic evaluation should be considered, even with normal fetal karyotype.

Oculoectodermal syndrome: twentieth described case with new manifestations

Abstract Oculoectodermal syndrome is a rare disease characterized by the association of aplasia cutis congenita, epibulbar dermoids, and other abnormalities. This report describes the twentieth case of the disease. We report a 4-year-old female child who presented with the classical features of the syndrome: aplasia cutis congenita and epibulbar dermoids. Our case expands the clinical spectrum of the disease to include: diffuse hyperpigmentation (some following the Blaschko´s lines); hypopigmented skin areas on the trunk; arachnoid cyst on the right fronto-parietal border; rounded left side of the hippocampus; and dermoid cyst underlying the bulb-medullary transition. Our patient also reported infantile hemangioma on the right wrist and verrucous hemangioma on the left leg, the latter not previously described in the literature.

A rare variant of ankyloblepharon filiforme adnatum associated with skin hypopigmentation: A case report from South India.

We report ankyloblepharon filiforme adnatum (AFA) along with associated skin lesions in a 7‑day‑old child from South India. It could be a variant of the well described ankyloblepharon‑ectodermal defects‑cleft lip and palate syndrome also called Hay–Wells syndrome wherein AFA, skin lesions, and clefting are characteristic. The ocular features, genetic inheritance, and possible systemic associations, along with the options for management, are discussed. The need for awareness among ophthalmologists of its systemic associations is discussed to ensure that proper multidisciplinary care is offered to the individuals affected by this rare disorder. This article also highlights the unusual hypopigmented skin lesions found in this infant, which has been scantily reported in the literature, as a possible variation in patients of Indian ethnicity.

Piebaldismo-Moebius y exposición prenatal a misoprostol: reporte de un caso / Piebaldism-Moebius and prenatal exposure to misoprostol: a case report / Piebaldismo-Moebius e exposição pré-natal a misoprostol: reporte de um caso

El piebaldismo es una enfermedad congénita rara de curso estático, con prevalencia de 1/100.000; se caracteriza por despigmentación en parches de la piel y presencia de un mechón blanco frontal. Por lo demás los pacientes son sanos. Se hereda con un patrón autosómico dominante. Es causado por mutaciones en el gen c-kit, un protoncogén que participa en la migración, proliferación, diferenciación y supervivencia de los melanoblastos. Por otro lado, el síndrome de Moebius es una parálisis congénita del VII par craneal, que puede estar asociada a compromiso de otros pares craneales, o incluso de otros sistemas. En Estados Unidos se ha calculado su frecuencia en 0,002 % - 0,0002 % del total de nacimientos. Presentamos el caso de una recién nacida con piebaldismo y síndrome de Moebius asociado a exposición prenatal a misoprostol. Se hace una búsqueda bibliográfica sobre las anomalías de la paciente y la asociación entre la exposición prenatal a misoprostol y anomalías congénitas. El piebaldismo es un trastorno raro de etiología genética. El síndrome de Moebius, en cambio, es de causa heterogénea y no bien definida, y se ha asociado a exposición prenatal a misoprostol. La paciente reportada presenta simultáneamente ambas entidades, una netamente genética y la otra posiblemente teratogénica.

Piebaldism is a rare congenital disease with prevalence of 1/100.000, characterized by patchy depigmentation of the skin and the presence of a white forelock. Its course is static and otherwise patients are healthy. It is inherited in an autosomal dominant pattern and is caused by mutations in the gene c-kit, a proto-oncogene involved in the migration, proliferation, differentiation and survival of melanoblasts. On the other hand, Moebius syndrome is a congenital palsy of the VII cranial nerve, which may be associated with involvement of other cranial nerves, or even of other systems. In the United States its frequency has been calculated from 0.002% to 0.0002% of total births. We report the case of a newborn girl with piebaldism and Moebius syndrome associated with prenatal exposure to misoprostol. A search was made about these anomalies and the association between prenatal exposure to misoprostol and congenital anomalies. Piebaldism is a rare genetic disorder. On the other hand, the cause of Moebius syndrome is heterogeneous and not well defined, and it has been associated with prenatal exposure to misoprostol. Our patient had simultaneously two diseases: one purely genetic and the other potentially teratogenic.

O piebaldismo é uma doença congênita esquisita de curso estático, com prevalência de 1/100.000; caracteriza- se por despigmentação em parches da pele e presença de uma mecha branca frontal. Pelo demais os pacientes são sãos. Herda-se com um padrão autossômico dominante. É causado por mutações no gene c-kit, um proto-oncogene que participa na migração, proliferação, diferenciação e sobrevivência dos melanoblastos. Por outro lado, a síndrome de Moebius é uma paralisia congênita do VII par craniano, que pode estar associada a compromisso de outros pares cranianos, ou inclusive de outros sistemas. Nos Estados Unidos se calculou sua frequência em 0,002 % - 0,0002 % do total de nascimentos. Apresentamos o caso de uma recém-nascida com piebaldismo e síndrome de Moebius associado a exposição pré-natal a misoprostol. Faz-se uma busca bibliográfica sobre as anomalias da paciente e a associação entre a exposição pré-natal a misoprostol e anomalias congênitas. O piebaldismo é um transtorno raro de etiologia genética. A síndrome de Moebius, em mudança, é de causa heterogênea e não bem definida, e se associou a exposição pré-natal a misoprostol. A paciente reportada apresenta simultaneamente ambas entidades, uma netamente genética e a outra possivelmente teratogênica.

Evaluation of Cosmetic Dermatitis in the Rohilkhand Region.

Background: Cosmetics have been used since the oldest known civilizations, and nowadays almost everybody resorts to beauty products.. Reactions to cosmetics constitute a small but significant number of cases of contact dermatitis that can present with varied appearances. Aims and objective: evaluate cosmetic dermatitis using cosmetics. Methods: The study was performed over a period of 6 months, starting in April 2015 in the Rohilkhand medical college and hospital. 200 patients were selected in skin OPD. The patients filled in a self-administered questionnaire concerning their use of cosmetics and skin care products and reported suspected current or previous adverse reactions to such products. Results: The patch test is done female are more adverse reaction than male. In female eye make 12% bindi 3% ,soap 2% tooth paste 4% hair dye 23% lipstick 4% manicure 6% deodrants 8% nail polish 3% nail polish remover is 6% and shampoo is 1% adverse reaction. Conclusion: cosmetics causing dermatitis. It is more common in female as compare to female.