Kutane und systemische Veränderungen mit Ähnlichkeiten zum Omenn‐Syndrom bei heterozygoter Mutation im RAG2‐Gen

Remisión espontánea de verrugas recalcitrantes en niñas tras vacunación frente al virus del papiloma humano

Parvovirus B19-Associated Microvesicular Eruption

We report on a 3-year-old girl with a microvesicular generalized rash in whom primary infection by parvovirus B19 was demonstrated by seroconversion. To our knowledge, this is the first instance of an eruption arising from parvovirus B19 with this peculiar clinical pattern.

Vascularización en dermatoscopia

Resumen La dermatoscopia permite observar, en las condiciones adecuadas, la vascularizacion presente en las lesiones cutaneas de muy diversa indole. La visualizacion e identificacion de vasos de morfologia caracteristica puede ser la clave en el diagnostico de determinadas lesiones, especialmente en el caso de aquellas hipopigmentadas, en las que no es posible identificar las estructuras pigmentadas clasicas. Asi pues, algunas de las asociaciones mas caracteristicas son la presencia de vasos «en corona» en la hiperplasia sebacea, las «telangiectasias arboriformes» en el carcinoma basocelular, los vasos «en coma» en los nevos intradermicos y compuestos, los vasos «en cabeza de alfiler» en lo…

An analysis of the stability of rural tourism as a desired condition for sustainable tourism

Abstract Tourism is a key sector in the sustainable development of rural environments. Its ability to create stable employment and an acceptable level of profits is conditioned by the stability of tourist activity throughout the year. This paper compares the level of seasonality of a group of rural destinations to that of coastal and urban destinations. By doing so, we intend to determine whether seasonality-related problems exist in the rural environment or not. The second aim is related to the first one: the proposal of a new, more comprehensive and objective methodology that can measure the intensity of seasonality based on a DP2 synthetic indicator. The DP2 indicator groups information …

BASCULE syndrome associated with presyncopal episodes and first‐degree atrioventricular block

Two-year-old girl with tuberous xanthomas.

A 2-year-old girl was referred for evaluation because she had two nodular lesions located on both heels, and another elongated lesion in the intergluteal cleft. On physical examination, two yellow to orange well-defined nodules, suggestive of xanthomas, were bilaterally located on the Achilles tendon areas (figure 1A). Moreover, another yellowish, slightly raised lesion with band-like morphology was seen in the intergluteal cleft (figure 1B). There were no other anomalies on physical examination. Figure 1 (A) Tuberous xanthoma located on the left heel. (B) Planar xanthoma located in the intergluteal cleft. (C) Peripheral blood smear examination showing numerous red cells and two macrothromb…

Dermoscopy of cutaneous smooth muscle neoplasms: a morphological study of 136 cases

BACKGROUND: A variety of cutaneous smooth muscle neoplasms may arise in the skin and are frequently unrecognized by clinicians. There is sparse data relating to the dermoscopy of piloleiomyomas (PL), and nothing has been published about the dermoscopy of angioleiomyomas (AL) and leiomyosarcomas (LS). OBJECTIVES: To evaluate the morphological findings of a large series of cutaneous PL, AL and LS under dermoscopic observation, comparing these findings among them. METHODS: Digital dermoscopic images of 136 histopathologically confirmed cases of cutaneous smooth muscle neoplasms (114 PL, 13 AL and 9 LS) collected from 10 Hospitals in Spain, Austria and Italy were evaluated for the presence of d…

Pápulas de coloración de la piel y malformación del tracto urinario. Diagnóstico y comentario

Azithromycin for the Treatment of Ciclosporin-Induced Gingival Hyperplasia

Respuesta favorable al tratamiento con alopurinol de reacción granulomatosa a relleno

Social Sustainability on Competitiveness in the Tourism Industry: Toward New Approach?

The achievements made over the years of policies seeking to stimulate tourism demand have led to a great increase in the number of tourists arriving in many destinations. Some destinations have begun to suffer problems derived from the success of policies focused solely on increasing the number of arrivals. The objective of this work is to analyze the role of social sustainability in the analysis of tourism competitiveness. Methodologically, this study has been developed from the analysis of papers published in journals indexed in the Journal Citations Reports and Scopus. This has been complemented with information extracted from a number of well-known studies regarding tourism competitiven…

Keratoacanthoma Centrifugum Marginatum (KCM) after photodynamic therapy with good response to oral retinoids and topical 5‐fluorouracil

Keratoacanthoma centrifugum marginatum (KCM) is a rare variant of keratoacanthoma (KA), characterized by progressive peripheral growth, and usually devoid of deep invasion. Different systemic (oral retinoids) or topical treatments have been reported, but there is not a well-defined therapeutic protocol. We report the case of a KCM developing after photodynamic therapy (PDT) on the right leg of a 64-year-old woman. It was treated successfully with oral acitretin combined with topical 5-Fluorouracil + salicylic acid for 5 months. This is the first case of KCM developing after PDT and successfully treated with oral retinoid combined with topical treatment.

Giant Nevus Lipomatosus Cutaneous Superficialis

Invasión de vasos linfáticos en nevos melanocíticos. Estudio clinicopatológico de ocho casos

Introduccion . La invasion de vasos linfaticos por melanocitos de nevos melanociticos es un hallazgo infrecuente. Por otro lado, la presencia de melanocitos en los ganglios linfaticos se detecta de forma casual en linfadenectomias regionales por carcinomas y melanomas. Metodos . Hemos estudiado 8 casos de nevos melanociticos cutaneos con invasion de vasos linfaticos, analizando sus caracteristicas clinicas e histologicas, asi como la inmunotincion para Ki-67. Resultados . Siete de los 8 casos correspondian a mujeres; cinco fueron diagnosticados como nevos congenitos, observandose en estos ultimos un crecimiento solido melanocitico intravascular con integridad de la superficie endotelial. En…

Leukocytoclastic vasculitis associated with sulfuric acid inhalation

A heterozygous mutation in the RAG2 gene with cutaneous and systemic manifestations partially resembling Omenn syndrome

Progressively fading asymptomatic brownish macules

Clinical, Microscopic and Ultrastructural Findings in a Case of Short Anagen Syndrome

Short anagen syndrome is an uncommon and recently described disease characterized by many telogen hairs and short maximum hair length. We report here the case of a 3-year-old girl whose short, sparse, fine hair since birth was consistent with short anagen syndrome. X-ray microanalysis demonstrated normal composition of the main bioelements of her hairs.

Familial Combined Hyperlipidemia, Metabolic Syndrome and Cardiovascular Disease

Our aim was to investigate the relationship between metabolic syndrome and cardiovascular disease (i.e., survivors of myocardial infarction) in patients with familial combined hyperlipidemia (FCH). We compared a group of 20 male patients with FCH who had survived a myocardial infarction with two other groups matched for age and body mass index, comprising 20 individuals with FCH who had not had a myocardial infraction and 20 control subjects. Plasma lipid, glucose, and insulin levels were determined. Metabolic syndrome was judged to present on the basis of World Health Organization (WHO) and National Cholesterol Education Program-Adult treatment panel (NCEP-ATPIII) criteria. Differences bet…

Progressive Orange Lesions on the Scalp

Pruriginous Lesions in a Young Girl: Challenge.

Novel Immunologic Approaches to Melanoma Treatment

Approaches to treating melanoma have changed radically since the introduction of immunotherapy, and survival figures are now higher than possible with earlier therapies. The immunomodulators currently available mainly block CTLA-4 (cytotoxicT lymphocyte-associated molecule-4) and PD-1 (programed cell death protein 1) translocated to the cell surface, where they inhibit the antitumor immune response. Treatments blocking these molecules are being more widely used. Research now seeks new molecular targets, the best combinations of available drugs, and biomarkers that can identify ideal candidates for each one.

Novedades en inmunología del melanoma

Resumen Desde la introduccion de la inmunoterapia el tratamiento del melanoma ha sufrido una revolucion, consiguiendose cifras de supervivencia superiores a las que se alcanzaban con los tratamientos previos. Los farmacos inmunomoduladores disponibles actualmente van dirigidos principalmente frente a las moleculas de superficie CTLA-4 y PD-1, que ejercen un efecto inhibidor sobre la respuesta inmune antitumoral. Se trata de un tratamiento en pleno proceso de expansion, y la investigacion se dirige hacia el descubrimiento de nuevas moleculas, las combinaciones de los farmacos disponibles o la identificacion de biomarcadores que permitan seleccionar a los pacientes idoneos para cada terapia.

Infantile hemangiomas with minimal or arrested growth: A retrospective case series

Background/objectives Infantile hemangiomas (IH) with minimal or arrested growth (MAG) constitute a distinctive subset of IH based on their clinical characteristics and natural history. They are often confused with capillary malformations. Methods A retrospective observational study has been carried out in which clinical and perinatal characteristics have been evaluated in all IH-MAG evaluated in our Dermatology Department in a 5-year period (January 2013-December 2017). Results A total of 14 IH-MAG affecting 13 patients were identified (10 girls and 3 boys). All were born full term, with a mean birth weight of 3448 g for girls vs 3540 g for boys, corresponding to the 72nd percentile for bo…

Blueberry Muffin Baby Associated with Bone Demineralization Due to Congenital Transient Neonatal Hyperparathyroidism

Lesiones anaranjadas de aparición progresiva en cuero cabelludo

Regression of multiple melanocytic nevi associated with nivolumab treatment for metastatic melanoma

Sedimentary model and high-frequency cyclicity in a Mediterranean, shallow-shelf, temperate-carbonate environment (uppermost Miocene, Agua Amarga Basin, Southern Spain)

Uppermost Tortonian to lower Messinian temperate carbonates crop out in the Agua Amarga Basin (SE Spain). They consist of four units. The lower three units can be tentatively assigned to the lowstand systems tract of a fourth-order sequence, constituting in turn the lowstand (‘megatrough unit’), transgressive (‘breccia unit’) and highstand (‘bedded unit’) stages of a higher-order cycle. All these materials were deposited in a small pull-apart basin related to the sinistral Carboneras strike-slip fault system. The best represented is the bedded unit (up to 25 m thick), which consists of bioclastic, bryozoan/bivalve-dominated calcarenites/calcirudites with abundant fragments of echinoids, bar…

Clinically atypical spitzoid lesions: semi-quantitative histologic index correlation with dermoscopic scores (ABCD rule, 7-point checklist and pattern analysis)

Background The distinction of Spitz and Reed nevi with atypical features from melanoma may be a difficult task. In these cases, the dermoscopic scores could bring variable results and not always correlate with the histologic diagnosis. Objective The aim of this study was to compare the histopathologic and dermoscopic findings in a cohort of clinically atypical Spitz and Reed nevi. Methods We retrospectively reviewed 19 cases of atypical Spitz and Reed nevi from our files. We constructed a new semi-quantitative histologic index (HI) based on the reproducible microscopic features, and correlated it with two dermoscopic scores: ABCD and 7-point checklist, as well as with the predominant dermos…

Trichoscopy features of trichotillomania

Trichotillomania is a form of traction alopecia resulting from repetitive and compulsive hair pulling and plucking. Trichotillomania and patchy alopecia areata may have similar clinical and dermoscopic features in some cases. On trichoscopic examination, the presence of black dots, coiled or hook hair, shafts of varying lengths with fraying or split ends (trichoptilosis), and an absence of exclamation mark hairs and yellow dots are suggestive of trichotillomania.

Spontaneous Remission of Recalcitrant Warts in Girls After Human Papillomavirus Vaccination

Síndrome de Gianotti-Crosti-like secundario a Molluscum contagiosum

Papel de las quimiocinas en la progresión del melanoma

Resumen Las metastasis son la principal causa de mortalidad en el melanoma. Las quimiocinas son citocinas quimiotacticas de bajo peso molecular que permiten la migracion celular, atrayendo a celulas que expresan sus receptores. Como algunas quimiocinas son abundantes en organos que son asiento comun de metastasis, y sus receptores son expresados por las celulas tumorales, se planteo la hipotesis de que un gradiente de estas quimiocinas facilitaria selectivamente las metastasis a esos organos. El hallazgo posterior de la produccion de estas quimiocinas por las propias celulas tumorales, y su efecto autocrino, obliga a modular esta hipotesis. Ademas, muchas quimiocinas tienen efectos contrapu…

Florid fibroplasia in melanocytic lesions. A retrospective study.

Depressed indurated plaque with elastorrhexis as a distinctive lesion in Buschke‐Ollendorff syndrome

Buschke-Ollendorff syndrome (BOS) is a rare autosomal dominant genodermatosis caused by heterozygous mutations in LEMD3 and characterized by connective tissue nevi and sclerotic bone lesions known as osteopoikilosis. We report a family with three individuals affected by BOS, two of whom manifested clinical and histopathological peculiarities, presenting with a depressed indurated plaque as the main cutaneous manifestation instead of the classic connective tissue nevi. Notable elastorrhexis was present in both biopsies.

Histiocytosis with mixed cell populations

Langerhans cell histiocytosis (LCH) and juvenile xanthogranuloma (JXG) are thought to originate from a common stem cell precursor, with divergent differentiation under different microenvironmental conditions. We describe an exceptional case of multiple cutaneous lesions in a 10-year-old boy, in which the coexistence of both LCH and JXG cell populations is found in every single lesion. The presence of Birbeck granules and CD207 (langerin) immunostaining in the LCH component would argue against the diagnosis of indeterminate cell histiocytosis (ICH). This unique case gives additional support to the hypothesis of a potentially common histogenesis for LCH and JXG.

The Usefulness of Dermoscopy for the Recognition of Malignant Collision Tumors.

<b><i>Background:</i></b> Preoperative diagnosis of malignant collision tumors (MCT) is extremely difficult. The value of dermoscopy to improve the correct detection of these tumors has not been previously studied. This study aims to evaluate the diagnostic accuracy of MCT with and without dermoscopy and to describe the dermoscopic features of a large series of MCT. <b><i>Methods:</i></b> Dermoscopic images of 161 MCT were evaluated. Clinical and dermoscopic images of histopathologically proven MCT intermingled with other tumors were randomly presented to clinicians with different levels of experience, blinded to the diagnosis and objective of…

Vascular Patterns in Dermoscopy

Under the right conditions, dermoscopy allows us to observe the vascular features of many different types of skin lesions. The visualization and identification of vessels with a characteristic morphology can be the key to diagnosis, especially in hypopigmented lesions in which the typical pigmented structures are not visible. Some of the more characteristic associations are the presence of crown vessels in sebaceous hyperplasia, arborizing telangiectasias in basal cell carcinoma, comma-shaped vessels in intra- dermal and compound nevi, dotted vessels in Spitz nevi and melanoma, and hairpin vessels in seborrheic keratoses. The recognition of distinctive vascular features can be of great help…

Facial cystic lesions and onychodystrophy.

Entrepreneurs' perceptions of official sporting events' capacity to promote their business in the long term

[EN] Academic literature includes numerous studies on the assessment of the economic impacts of sporting events. Some of these impacts are only temporary and don't produce a lasting effect on the productive fabric, while others lead to long term developments. Even though residents' reactions to the long term impacts of these events have been broadly studied, the views expressed by entrepreneurs located in the area of the event remain to be investigated. This article analyses perceptions of entrepreneurs located near the Sierra Nevada Ski and Mountain Resort in Spain relating to their assessment of the long term effects of hosting the 2017 Snowboard and Freestyle Ski World Championship.This …

Nearshore, temperate, carbonate depositional systems (lower Tortonian, Agua Amarga Basin, southern Spain): implications for carbonate sequence stratigraphy

Abstract The bryozoan-rich lower Tortonian carbonates of the Agua Amarga Basin in southern Spain (Province of Almeria) provide an example of sediments formed in a nearshore, non-tropical depositional setting. Based on data derived from logging of sections and from field mapping, these lower Tortonian carbonates form a depositional sequence, which is subdivided into several depositional systems. A lowstand systems tract, which consists of volcaniclastic fan deltas and washover deposits, formed on the leeward side of a basement shoal which delimited the basin towards the south. A transgressive systems tract, which is characterised by a landward encroachment of deposits, is represented by subm…

Circulating miRNA expression analysis reveals new potential biomarkers for human cutaneous melanoma staging.

Telomeric length heterogeneity influences spontaneous regression of malignant melanoma

Pápulas de coloración de la piel y malformación del tracto urinario

Role of Chemokines in Melanoma Progression

Metastasis is the main cause of death from melanoma. Chemokines are low molecular weight chemotactic cytokines that facilitate cellular migration. Thus, cells that express receptors for a given chemokine are attracted to the site of its production. As certain chemokines are found in abundance in organs that are common targets of metastasis and receptors for these chemokines are expressed by tumor cells, it was hypothesized that chemokine gradients might selectively facilitate metastasis to these organs. A later finding that these chemokines were produced by tumor cells, with evidence of autocrine effects, obliged the modification of that hypothesis. Many chemokines are also known to have op…

Estudio retrospectivo de hemangiomas infantiles: Características clínicas, perinatales y factores predictores del desarrollo de secuelas residuales

Resumen Introduccion El hemangioma infantil (HI) es el tumor mas frecuente de la infancia. Es un tumor benigno de origen vascular, que afecta a la edad pediatrica exclusivamente. La mayoria sigue un curso clinico benigno y se resuelve sin necesidad de tratamiento, aunque con frecuencia origina lesiones residuales una vez resuelto. El objetivo de este estudio fue realizar un estudio descriptivo de los HI evaluados en un hospital terciario, evaluar la incidencia de lesiones residuales y predecir los factores relacionados con el desarrollo de las mismas. Material y metodos Se realizo un estudio observacional retrospectivo recogiendo los HI de los que se disponia de imagenes clinicas evaluados …

Hiperlipidemia familiar combinada, síndrome metabólico y enfermedad cardiovascular

Se estudia la relacion entre sindrome metabolico (SM) e infarto agudo de miocardio (IAM) en la hiperlipidemia familiar combinada (HFC). Se comparan 20 sujetos varones con HFC supervivientes a IAM con otras 2 series de sujetos emparejados por edad e indice de masa corporal (IMC): 20 individuos con HFC que no han presentado IAM y 20 controles sanos. Se determinaron los lipidos, la glucosa y la insulina en plasma y la presencia de SM definido por criterios de la Organizacion Mundial de la Salud (OMS) y National Cholesterol Education Program-Adults Treatment Panel (NCEP-ATP-III). El SM definido por criterios OMS se encontro en 19 sujetos con HFC e IAM, en 11 sujetos con HFC sin IAM y en 6 contr…

Spontaneous Regression of a Recalcitrant Wart after Bivalent Papillomavirus Vaccination

Painful papule on the right arm of a woman.

Foreign-Body Reaction to Dermal Filler: Good Response to Treatment With Allopurinol

Bascule syndrome associated with syncopal episodes.

Bascule syndrome is a recently described benign vasomotor dermatosis characterized by Bier anemic spots, cyanosis, and urticaria-like eruption. We report a case of a 13-year-old girl with cutaneous lesions consistent with Bascule syndrome who had had three exercise-related syncopal episodes. It would be recommended to exclude orthostatic intolerance or postural orthostatic tachycardia syndrome when evaluating patients with Bascule syndrome.

Ichthyosis, atopic dermatitis, and alopecia