Dyskeratosis congenita associated with a novel missense variant in TERT: Approach for the dermatologists.

Dyskeratosis congenita (DC) is a telomeropathy presenting diagnostic and therapeutic challenges across multiple specialties; yet, subtle dermatological signs enable early detection, altering patient prognosis. A specific DC genetic sequencing was performed according to the clinical criteria of our patient in study. Subsequently, cross-checked information in the main genetic databases was carried out. Additionally, an extensive review of the literature was made to organize the main dermatological aspects in DC. We report a novel variant of DC. Additionally, we share 10 useful and practical messages for dermatologists and any specialist caring for this group of patients.

Metastatic Crohn's disease: a difficult diagnosis from different perspectives

Metastatic Crohn's disease (MCD) is a rare cutaneous manifestation of Crohn's disease (CD). The simultaneous involvement of the vulva and oral region is uncommon in clinical presentations of MCD. We present the case of a middle-aged woman with a family history of autoimmunity who initially presented with oral and vulvoperineal involvement. Initially, Behçet's disease was ruled out, but histopathological studies of the vulva revealed findings compatible with MCD. The patient had no gastrointestinal symptoms, and fecal calprotectin levels were normal. Upper and lower endoscopic examinations and capsule endoscopy of the small intestine (SI) did not reveal any significant findings. Treatment with anti-tumor necrosis factor (anti-TNF) agents was initiated but resulted in paradoxical psoriasis with adalimumab and infliximab. Cyclosporine was also used, but the patient experienced intolerable tachycardia. After 18 months, the patient developed episcleritis and experienced diarrhea accompanied by cramp-like abdominal pain. Repeat upper and lower endoscopic examinations showed normal results, while capsule endoscopy of the SI revealed CD enteritis. The patient was diagnosed with CD of the small intestine, along with extraintestinal manifestations of vulvoperineal MCD, oral involvement, and episcleritis. Management with azathioprine and ustekinumab was initiated, resulting in significant clinical improvement. MCD poses a diagnostic challenge due to its unusual manifestations. It may present without gastrointestinal tract involvement, mimicking other conditions. Therefore, timely diagnosis and the selection of the most appropriate therapeutic strategy are crucial.

La enfermedad de Crohn metastásica (ECM) es una entidad rara, es una manifestación cutánea en enfermedad Crohn (EC), y en su presentación clínica es inusual el compromiso vulvar y oral de modo concomitante. Se describe el caso de una mujer de edad media que tiene historia familiar de autoinmunidad, presenta inicialmente compromiso oral y vulvoperineal. En su abordaje inicial se descartó enfermedad de Behçet, pero los estudios histopatológicos a nivel vulvar tuvieron hallazgos compatibles para ECM. Hubo ausencia de síntomas gastrointestinales y sus niveles de calprotectina fecal eran normales. Los estudios endoscópicos altos y bajos, y la cápsula endoscópica del intestino delgado (ID) no tuvieron hallazgos relevantes. Se inició el tratamiento con antifactor de necrosis tumoral (anti-TNF) y presentó una psoriasis paradójica con adalimumab e infliximab. También se utilizó ciclosporina y presentó una taquicardia intolerable. 18 meses después de estos síntomas tuvo epiescleritis e inició con diarrea asociada a dolor abdominal tipo cólico, por lo que se repitieron los estudios endoscópicos altos y bajos, que resultaron normales, y cápsula endoscópica de ID mostró una enteritis por EC. Se consideró EC de ID con manifestaciones extraintestinales y con ECM vulvoperineal, compromiso oral y epiescleritis. Se requirió manejo con azatioprina y ustekinumab, con una mejoría clínica significativa. La ECM es un reto diagnóstico, pues es una manifestación inusual; en su debut puede haber ausencia de compromiso en el tracto gastrointestinal y también simular otras entidades. Debe buscarse lograr el diagnóstico oportuno y la estrategia terapéutica más segura.

Cutaneous Manifestations in Patients With COVID-19 Treated at a University Hospital in Southern Brazil.

Objectives The aim of this study was to ascertain whether pattern of cutaneous lesions, age, sex, ethnicity, long-term medication use, arterial oxygen saturation at the first examination, setting of care, and number of medications used to treat SARS-CoV-2 infection are associated with mortality in patients with a confirmed diagnosis of coronavirus disease 2019 (COVID-19) and cutaneous manifestations. In addition, to evaluate the occurrence of cutaneous manifestations in patients with a confirmed diagnosis of COVID-19 through a review of medical records and in-person evaluation by a dermatologist. Methods This investigation consisted of two components - (A) a cross-sectional study with a retrospective review of the medical records of all patients with a positive reverse-transcriptase polymerase chain reaction (RT-PCR) test for SARS-CoV-2 treated at Santa Casa de Misericórdia de Porto Alegre between March 2020 and November 2020, and (B) a prospective case series with in-person skin examination by an attending dermatologist of all patients admitted to COVID-19 wards between April 2021 and July 2021. The pattern of skin lesions and other variables were assessed. Results Information from 2968 individuals with COVID-19 was collected (2826 from the medical records and 142 from the in-person examination by a dermatologist). Of these, a total of 51 patients (1.71%) had COVID-19-related cutaneous lesions - 36 from the medical records group (1.27% of cutaneous manifestations) and 15 from the examinated group (10.56% of cutaneous manifestations). Of 51 patients, 15 (29.41%) died. There was no association between mortality and patterns of cutaneous manifestations. The variables male sex (p=0.021), intensive care unit (ICU) admission (p=0.001), and use of three or more antibiotics (p=0.041) were associated with higher mortality. Conclusions The risk factors, proven by our study, for mortality in patients with COVID-19 and cutaneous manifestations were male sex, ICU stays, and use of three or more antibiotics. Using the review of medical records as a tool for evaluating cutaneous manifestations related to COVID-19, there are about 10 times fewer occurrences when compared to in-person evaluation by a dermatologist.

[Urticaria associated with COVID-19 in allergic patients. A case series]. / Urticaria asociada a COVID-19 en pacientes alérgicos. Serie de casos.

INTRODUCTION: In December 2019, a new coronavirus was identified as the causal agent of the 2019 coronavirus disease (COVID-19) with a wide clinical spectrum that goes from asymptomatic cases to critical and fatal cases. Its usual manifestations are fever, cough, and fatigue; although some cases present skin lesions. CASES REPORT: The first case was a 30-year-old woman with a history of controlled solar urticaria who presented erythematous and pruritic wheals in malar and cervical regions and extremities; associated with general malaise, headaches, and nausea, in whom the presence of COVID-19 was confirmed; her condition improved with a quadruple dose of antihistamines. The second case was a 47-year-old woman with a history of wasp sting anaphylaxis, without mastocytosis, who presented nasal congestion, headaches, and hypogeusia associated with generalized, erythematous, and pruritic wheals, with confirmation of COVID-19; her condition improved with quadruple doses of antihistamines. CONCLUSIONS: Cutaneous manifestations associated with COVID-19 are frequent and can be classified as inflammatory and vascular. Among them, urticarial lesions occupy the fourth place, and although there could be some predominance of these in patients with a history of allergies, more studies are required to define an association.

Introducción: En diciembre de 2019 fue identificado un nuevo coronavirus como agente causal de la enfermedad por coronavirus del 2019 con un amplio espectro clínico, desde casos asintomáticos hasta críticos y fatales. Generalmente se manifiesta con fiebre, tos y fatiga, aunque algunos casos se presentan con lesiones cutáneas. Reporte de casos: El primer caso es una mujer de 30 años con antecedente de urticaria solar controlada quien presentó habones eritematosos y pruriginosos en región malar, cervical y extremidades, asociado a malestar general, cefalea y náuseas, en quien se confirmó la presencia de COVID-19, y mejoró con antihistamínicos a dosis cuádruple. El segundo caso es una mujer de 47 años con antecedente de anafilaxia por picadura de avispa, sin mastocitosis, quien presentó congestión nasal, cefalea e hipogeusia asociado a habones generalizados, eritematosos y pruriginosos, con confirmación de COVID-19, y mejoría con dosis cuadruple de antihistamínicos. Conclusiones: Las manifestaciones cutáneas asociadas a COVID-19 son frecuentes y se pueden clasificar en inflamatorias y vasculares. Dentro de ellas, las lesiones urticariales ocupan el cuarto lugar, y aunque podría existir algún predominio de estas en pacientes con antecedentes de alergias, se necesitan más estudios para definir una asociación.

Sarcoidosis cutánea: preámbulo de la enfermedad sistémica / Cutaneous sarcoidosis: Preamble of the systemic disease

RESUMEN La sarcoidosis es una enfermedad granulomatosa, multisistémica, de causa desconocida y con múltiples manifestaciones clínicas. La característica histopatológica esencial son los granulomas uniformes no caseificantes con escasos linfocitos periféricos (granulomas desnudos). Se presenta el caso de un paciente masculino de 62 años que acudió a Cuerpo de Guardia por presentar tos seca, falta de aire, inflamación de ganglios axilares y lesiones en piel, en forma de placas, máculas y otras de aspecto psoriasiforme. Las manifestaciones clínicas, los resultados de los exámenes complementarios y al hacer el diagnóstico diferencial con otras afecciones que cursan con hallazgos similares, se llegó al diagnóstico de sarcoidosis sistémica. El paciente evolucionó favorablemente con el tratamiento indicado. El compromiso cutáneo de la sarcoidosis representa un desafío dada la variada presentación de esta entidad y los diagnósticos diferenciales a considerar.

ABSTRACT Sarcoidosis is a multisystemic granulomatous disease of unknown etiology with multiple clinical manifestations. The essential histopathologic feature is uniform non-caseating granulomas with few peripheral lymphocytes (naked granulomas). We present the case of a 62-year-old male patient who came to the emergency room due to a dry cough, shortness of breath, swollen axillary glands and skin lesions, in the form of plaques, macules, and others with a psoriasiform appearance. The clinical manifestations, the results of the complementary tests and when making the differential diagnosis with other conditions that present similar findings, the diagnosis of systemic sarcoidosis was reached. The patient evolved favorably with the indicated treatment. The cutaneous involvement of sarcoidosis represents a challenge given the varied presentation of this entity and the differential diagnoses to be considered.

Skin manifestations in pediatric patients with primary immunodeficiency diseases (PIDs) in a tertiary care hospital in Colombia.

BACKGROUND: The current literature describes the characteristics of some skin manifestations in the context of primary immunodeficiency diseases (PIDs), also known as inborn errors of the immune system. However, there are hardly any data on the epidemiological trends of skin manifestations and PIDs in Latin America (LA). We aimed to describe the characteristics of patients with skin manifestations and the diagnosis of a PID treated at a tertiary hospital in Colombia. METHODS: This was a retrospective observational study. Data were taken from the institutional database of pediatric PIDs, which includes 306 patients under 18 years of age who attended a tertiary care center in Cali, Colombia for inpatient or outpatient services between December 2013 and December 2018. A trained third-year dermatology resident reviewed the electronic clinical records of all the patients in the database and double-checked patients who presented with cutaneous signs and symptoms. RESULTS: A total of 83 patients out of the original 306 patients (27.1%) presented with some type of cutaneous manifestation. Of these patients, 56.6% had atopic dermatitis, 56.6% reported at least one episode of skin infection, and some of the patients had both of these manifestations. Infections were more frequent in the PID group of combined immunodeficiency associated with well-defined syndromes and atopic dermatitis in the group of antibody deficiencies. CONCLUSIONS: It is important to recognize dermatological clinical characteristics in patients with PIDs. More studies are necessary to establish recommendations regarding the approach of diagnosis and management of these patients.

Dermatological aspects of SARS-CoV-2 infection: mechanisms and manifestations.

The human infection caused by the novel SARS-CoV-2 is a public health emergency of international concern. Although the disease associated to this virus, named COVID-19, mainly affects the lungs, the infection can spread to extrapulmonary tissues, causing multiorgan involvement in severely ill patients. The broad infective capacity of SARS-CoV-2 is related to the pattern of expression of the viral entry factors ACE2 and TMPRSS2 in human tissues. As such, the respiratory and gastrointestinal tracts are at high risk for SARS-CoV-2 infection due to their high expression of ACE2 and TMPRSS2, which explains the clinical phenotype described in the vast majority of infected patients that includes pneumonia and diarrhea. Recently, preoccupation about the potential of the virus to infect the skin has been raised by dermatologists due to the increasing observations of cutaneous manifestations in patients with SARS-CoV-2 infection. Although there is little evidence of the expression of ACE2 and TMPRSS2 in the normal skin, the dermatological findings observed among COVID-19 patients warrants further investigation to delineate the mechanisms of skin affection after SARS-CoV-2 infection. Here, we provide a summary of the dermatological findings observed among patients with laboratory-confirmed SARS-CoV-2 infection based on recent reports. In addition, we analyze possible mechanisms of skin injury in COVID-19 patients and discuss about the risk of individuals with chronic skin conditions for SARS-CoV-2 infection. The present review constitutes a useful informative tool to improve our understanding of the pathophysiological mechanisms of COVID-19 and the possible implications of the current pandemic in dermatology.

Compromiso cutáneo secundario al uso del equipo de protección contra COVID-19 en el personal de la salud / Secondary skin manifestations on the use of COVID-19 protection equipment in health professional

Resumen Desde la declaración de pandemia del coronavirus el mundo entero ha encaminado sus esfuerzos a ayudar a mitigar sus efectos, especialmente el personal de la salud. En ese intento por ayudar, buscan evitar su propio contagio por medio del uso de materiales de protección como mascarillas, gafas y guantes. Aunque protectores, éstos materiales y sus componentes pueden ser altamente perjudiciales para la piel, causando dermatitis de contacto, ulceraciones y erosiones. En esta revisión se abordan dichas manifestaciones y se proponen medidas para prevenirlas o tratarlas.

Abstract Since the declaration of coronavirus as a pandemic the entire world has directed its efforts to help mitigate the effects of it to the best of its ability, especially health personnel. In that attempt to help, we seek to avoid their own contact through the use of protective materials such as masks, goggles, and gloves. Although protective, this equipmment and its components can be highly damaging to the skin, causing contact dermatitis. This review addresses manifestations and reviews measures to prevent and cure them.

Cutaneous susceptibility to SARS-CoV-2 infection according to the expression of viral entry factors in the skin.

INTRODUCTION: Reports of dermatological manifestations in patients with COVID-19 suggest a possible cutaneous tropism of SARS-CoV-2; however, the capacity of this virus to infect the skin is unknown. OBJECTIVE: To determine the susceptibility of the skin to SARS-CoV-2 infection based on the expression of viral entry factors ACE2 and TMPRSS2 in this organ. METHOD: A comprehensive analysis of human tissue gene expression databases was carried out looking for the presence of the ACE2 and TMPRSS2 genes in the skin. mRNA expression of these genes in skin-derived human cell lines was also assessed. RESULTS: The analyses showed high co-expression of ACE2 and TMPRSS2 in the gastrointestinal tract and kidney, but not in the skin. Only the human immortalized keratinocyte HaCaT cell line expressed detectable levels of ACE2, and no cell line originating in the skin expressed TMPRSS2. CONCLUSIONS: Our results suggest that cutaneous manifestations in patients with COVID-19 cannot be directly attributed to the virus. It is possible that cutaneous blood vessels endothelial damage, as well as the effect of circulating inflammatory mediators produced in response to the virus, are the cause of skin involvement.

INTRODUCCIÓN: Reportes de manifestaciones dermatológicas en pacientes con COVID-19 sugieren un posible tropismo cutáneo del virus SARS-CoV-2; sin embargo, se desconoce la capacidad de este virus para infectar la piel. OBJETIVO: Determinar la susceptibilidad de la piel a la infección por SARS-CoV-2 con base en la expresión de los factores de entrada viral ACE2 y TMPRSS2 en dicho órgano. MÉTODO: Se buscaron los genes ACE2 y TMPRSS2 en la piel, para lo cual se realizó un análisis extenso de las bases de datos de expresión genética en tejidos humanos. Asimismo, se evaluó la expresión de dichos genes en líneas celulares humanas derivadas de la piel. RESULTADOS: Los análisis mostraron alta expresión conjunta de ACE2 y TMPRSS2 en el tracto gastrointestinal y en los riñones, pero no en la piel. Solo la línea celular de queratinocitos humanos inmortalizados HaCaT expresó niveles detectables de ACE2 y ninguna línea celular de origen cutáneo expresó TMPRSS2. CONCLUSIONES: Los resultados sugieren que las manifestaciones dermatológicas en pacientes con COVID-19 no pueden ser atribuidas directamente al virus; es posible que sean originadas por el daño endotelial a los vasos sanguíneos cutáneos y el efecto de los mediadores inflamatorios circulantes producidos en respuesta al virus.

Clinical and histopathological findings of cutaneous manifestations of COVID-19 patients.

Coronavirus disease (COVID-19) became pandemic since this is a disease with a high infection rate. The study's object is to identify clinical and histopathological findings of cutaneous manifestations of COVID-19 patients. The evidence was analyzed in PubMed, CINAHL, Cochrane, Web of Science, and Scopus, with the search strategy (covid 19 OR covid-19 OR corona OR coronavirus OR sars-cov-2) AND (cutaneous or cutaneous manifestations OR), and 17 studies were included, involving 351 cases with COVID19 and skin manifestations. The lesions were classified mainly as maculopapular, erythematous, vesicular, and urticarial. Histopathological analyses suggest a predominance of spongiosis, perivascular infiltrate of lymphocytes, and thrombogenic vasculopathy.

Cutaneous susceptibility to SARS-CoV-2 infection according to the expression of viral entry factors in the skin / Susceptibilidad cutánea a la infección por SARS-CoV-2 según la expresión de los factores de entrada viral en la piel

Abstract Introduction: Reports of dermatological manifestations in patients with COVID-19 suggest a possible cutaneous tropism of SARS-CoV-2; however, the capacity of this virus to infect the skin is unknown. Objective: To determine the susceptibility of the skin to SARS-CoV-2 infection based on the expression of viral entry factors ACE2 and TMPRSS2 in this organ. Method: A comprehensive analysis of human tissue gene expression databases was carried out looking for the presence of the ACE2 and TMPRSS2 genes in the skin. mRNA expression of these genes in skin-derived human cell lines was also assessed. Results: The analyses showed high co-expression of ACE2 and TMPRSS2 in the gastrointestinal tract and kidney, but not in the skin. Only the human immortalized keratinocyte HaCaT cell line expressed detectable levels of ACE2, and no cell line originating in the skin expressed TMPRSS2. Conclusions: Our results suggest that cutaneous manifestations in patients with COVID-19 cannot be directly attributed to the virus. It is possible that cutaneous blood vessels endothelial damage, as well as the effect of circulating inflammatory mediators produced in response to the virus, are the cause of skin involvement.

Resumen Introducción: Reportes de manifestaciones dermatológicas en pacientes con COVID-19 sugieren un posible tropismo cutáneo del virus SARS-CoV-2; sin embargo, se desconoce la capacidad de este virus para infectar la piel. Objetivo: Determinar la susceptibilidad de la piel a la infección por SARS-CoV-2 con base en la expresión de los factores de entrada viral ACE2 y TMPRSS2 en dicho órgano. Método: Se buscaron los genes ACE2 y TMPRSS2 en la piel, para lo cual se realizó un análisis extenso de las bases de datos de expresión genética en tejidos humanos. Asimismo, se evaluó la expresión de dichos genes en líneas celulares humanas derivadas de la piel. Resultados: Los análisis mostraron alta expresión conjunta de ACE2 y TMPRSS2 en el tracto gastrointestinal y en los riñones, pero no en la piel. Solo la línea celular de queratinocitos humanos inmortalizados HaCaT expresó niveles detectables de ACE2 y ninguna línea celular de origen cutáneo expresó TMPRSS2. Conclusiones: Los resultados sugieren que las manifestaciones dermatológicas en pacientes con COVID-19 no pueden ser atribuidas directamente al virus; es posible que sean originadas por el daño endotelial a los vasos sanguíneos cutáneos y el efecto de los mediadores inflamatorios circulantes producidos en respuesta al virus.

Lesiones en piel en pediatría en periodos de COVID-19. Reporte de un caso

RESUMEN En noviembre del 2019 inicia en Wuhan, lo que sería una nueva pandemia, por un coronavirus que no había sido identificado previamente, el SARS-CoV-2. Posteriormente fue reportado en Paraguay el primer caso de covid-19 en marzo del 2020. A pesar de que se trata de un virus de manifestación preferentemente respiratoria, sabemos hasta la fecha que pueden presentar otros síntomas, incluso ser totalmente asintomáticos. Se ha visto que las lesiones en piel, al igual que en muchas otras enfermedades infecciosas, también son causadas por este nuevo virus, por lo que el dermatólogo juega un rol importante en esta batalla. Reportamos el primer caso registrado por el servicio de Dermatología del Hospital Nacional de Itaugua, de manifestaciones cutáneas en un lactante, secundarias a la enfermedad de COVID-19.

ABSTRACT In November 2019, in Wuhan, China, what would be a new pandemic started, due to a coronavirus that had not been previously identified, SARS-CoV-2. Subsequently, the first case of COVID-19 was reported in Paraguay in March 2020. Despite the fact that it is a virus of preferential respiratory manifestation, we know to date that it can present with other symptoms, including asymptomatically. Skin lesions, like in many other infectious diseases, have been shown to be caused by this new virus as well, making the dermatologist play an important role in this battle. We report the first case registered by the Dermatology service of the Itaugua National Hospital of cutaneous manifestations in an infant, secondary to COVID-19 disease.

Manifestaciones extrapulmonares de la infección por el nuevo Coronavirus SARS-CoV-2 / Extra-pulmonary manifestations of the novel coronavirus infection SARS-CoV-2

Introducción: Los Coronavirus son una extensa familia de virus que pueden causar enfermedades tanto en animales como en humanos. El SARS-CoV-2 es un nuevo virus que pertenece a la subfamilia Orthocoronavirinae. La infección puede ser leve, moderada o severa en correspondencia con la forma de presentación y los síntomas que la acompañan. Cuando no hay complicaciones, la infección vírica de las vías respiratorias altas cursa con síntomas inespecíficos como fiebre, tos, dolor de garganta, congestión nasal, malestar general, cefaleas y dolores musculares. Objetivo: Describir las manifestaciones extrapulmonares de la infección por el nuevo coronavirus SARS-CoV-2. Material y Métodos: Se realizó una revisión bibliográfica a partir de un total de 56 referencias; se utilizaron artículos e información en idioma español e inglés disponibles en bases de datos como PubMed/MedLine, SciELO, Scopus y otras revistas de acceso abierto. Desarrollo: Diferentes autores han reportado manifestaciones extrapulmonares en pacientes enfermos con la COVID-19. En el sistema digestivo se han reportado, entre otras, náuseas, vómitos, diarrea y lesión hepática; en el sistema cardiovascular hipotensión arterial, arritmias, miocarditis, y otras; en el sistema renal se ha observado injuria renal aguda; en el sistema endocrinometabólico la hiponatremia se observó en un caso reportado; en el sistema neurológico se han observado epilepsia, anosmia, enfermedades cerebrovasculares, entre otras; además se han observado manifestaciones cutáneas como lesiones exantemáticas. Conclusiones: La infección por el nuevo Coronavirus SARS-CoV-2 además de las manifestaciones pulmonares típicas de la neumonía que provoca, presenta manifestaciones extrapulmonares de los sistemas: digestivo, cardiovascular, endocrinometabólico, neurológico, renal y manifestaciones cutáneas(AU)

Introduction: Coronaviruses are a large family of viruses that can cause diseases in both animals and humans. The SARS-CoV-2 is a new virus that belongs to the Orthocoronavirinae subfamily. The infection can be mild, moderate or severe regarding the form of presentation and the accompanying symptoms. When there are no complications, the viral infection of the upper respiratory tract is accompanied by nonspecific symptoms like fever, cough, sore throat, nasal congestion, general malaise, headaches and muscle pain. Objective: To describe the extra-pulmonary manifestations of the novel coronavirus infection SARS-CoV-2. Material and Methods: A bibliographic review from a total of 56 references was made; articles and information in Spanish and English languages available from databases such as PubMed/MedLine, SciELO, Scopus and other open access journals were used. Development: Different authors have reported extra-pulmonary manifestations in patients with COVID-19. Nausea, vomiting, diarrhea and hepatic lesions among others have been reported in the digestive system; arterial hypotension, arrhythmias and myocarditis have been found in the cardiovascular system; acute kidney injury has been observed in the renal system; hyponatremia was reported in a case in the endocrine-metabolic system; epilepsy, anosmia, cerebrovascular disease and others have been observed in the neurological system; additionally, cutaneous manifestations have been observed as exanthematic lesions. Conclusions: Besides the lung manifestations typical of pneumonia that the novel coronavirus infection SARS-CoV-2 causes, extra-pulmonary manifestations can be present in other systems such as: the digestive, cardiovascular, endocrine-metabolic, neurological, and renal ones. Skin lesions can also occur(AU)

Manifestaciones cutáneas en pacientes pediátricos infectados por el coronavirus SARS-CoV-2 / Cutaneous manifestations in pediatric patients infected with SARS-CoV-2 coronavirus

Introducción: El nuevo coronavirus SARS-CoV-2 ha causado un brote grave de la enfermedad denominado COVID-19. El virus fue identificado por primera vez en Wuhan, China, en diciembre del 2019. Recientemente se describieron las manifestaciones cutáneas, con similitud clínica entre niños y adultos. Se identificaron cinco patrones cutáneos: erupción máculo-papular, urticaria, lesiones acrales tipo perniciosas, erupción vesicular y livedo reticularis. Objetivo: Caracterizar a pacientes pediátricos con manifestaciones cutáneas en el curso de la COVID-19. Presentacion de casos: Se estudiaron 10 pacientes pediátricos con COVID-19 ingresados en el Hospital Militar de Villa Clara Comandante Manuel Piti Fajardo. Del total de pacientes, 8 presentaron rash eritemato-papuloso y 2 rash urticariforme. Las lesiones urticariformes se presentaron solamente en las féminas y las eritemato-papulosas, de forma similar en ambos sexos con ligero predominio en las adolescentes pacientes femeninas. Ambas lesiones predominaron en el tronco y acompañadas de un prurito ligero o ausente. Solo requirieron tratamiento médico las lesiones urticariformes. Conclusiones: La principal expresión clínica en la población pediátrica atendida en Villa Clara son las manifestaciones cutáneas. Las lesiones eritemato-papulosas son las más frecuentes, tienen. aparición tardía y evolución favorable sin necesidad de tratamiento y sin acompañarse de otra sintomatología(AU)

Introduction: The new coronavirus called SARS-Cov-2 has caused a severe outbreak of the disease called COVID-19. This virus was identified for the first time in Wuhan, China on December,2019. There were recently described its cutaneous manifestations, with clinical similarities among children and adults. There were identified five cutaneous patterns: macular-papular rash, hives, acral lesions pernicious type, vesicular rash and livedo reticularis. Objective: To characterize pediatric patients with cutaneous manifestations during COVID-19 disease. Cases presentation: There were studied 10 pediatric patients with COVID-19 admitted in ´´Comandante Manuel Piti Fajardo´´ Military Hospital of Villa Clara province. From the total of patients, eight presented eritemato-papular rash and two had urticariform rash. Urticariform lesions were present just in the girls, the eritomato-papulosa lesions presented similarly in both sexes with slight predominance in female adolescents. Both types of lesions appeared with predominance in the torso and accompanied of slight or absent pruritus. Just the urticariform lesions required medical treatment. Conclusions: The main clinical presentations in the pediatric population attended in the Military Hospital of Villa Clara province are the cutaneous manifestations. Eritemato-papular lesions are the most frequent; they have late onset and favorable evolution with no need of treatment and with no other symptomatology(AU)

Manifestaciones cutáneas extraglandulares en pacientes con síndrome de Sjögren primario / Extraglandular cutaneous manifestations in patients with primary Sjögren's syndrome

RESUMEN Objetivos: Describir la frecuencia de manifestaciones cutáneas extraglandulares en pacientes con síndrome de Sjögren primario. Determinar el perfil clínico y de laboratorio de los pacientes que presentan estas manifestaciones en comparación con aquellos que no las presentan. Materiales y métodos: Se analizaron los datos de los pacientes incluidos en la base GESSAR (Grupo de Estudio Síndrome de Sjögren de la Sociedad Argentina de Reumatología). Para la comparación entre grupos, los controles se seleccionaron en forma aleatoria con una relación casos: controles de 1:4. A su vez, se compararon los pacientes con púrpura con los controles. Resultados: Sesenta y siete (14,1%) de los 474 pacientes incluidos en la base de datos tuvieron manifestaciones cutáneas extraglandulares. De ellos, el 58% tuvo púrpura. La artritis, la neuropatía, el descenso de C3 y de C4, y la crioglobulinemia fueron estadísticamente más frecuentes en los casos en comparación con los controles; sin embargo, no se encontró asociación independiente con ninguna de estas variables. En lo que respecta a púrpura, la artritis, la neuropatía periférica, la anemia, el descenso de C3 y de C4, anti-La y crioglobulinemia fueron estadísticamente más frecuentes en comparación con los controles. Solo el descenso de C4 y la positividad de crioglobulinas se asociaron en forma independiente a la presencia de púrpura. Conclusión: El 14% de los pacientes presentaron manifestaciones cutáneas extraglandulares. La púrpura fue la manifestación más frecuente. Esta se asoció en forma independiente con el descenso de C4 y la presencia de crioglobulinas.

ABSTRACT Objectives: To describe the frequency of extra-glandular cutaneous manifestations in patients with primary Sjögren's syndrome. To determine the clinical and laboratory profile of patients who present with these manifestations compared to those who do not. Materials and methods: A study was made of patients included in GESSAR database (Sjögren Syndrome Society of Argentina Rheumatology Study Group) were analyzed. For the comparison between groups, the controls were randomly selected, with a case:control ratio of 1:4. Patients with purpura were compared with controls. Results: A total of 67 (14.1%) of the 474 patients included in the database had extra-glandular cutaneous manifestations. Of them, 58% had purpura. Arthritis, neuropathy, a decrease in C3 and C4 levels, and the presence of cryoglobulins, were statistically more frequent in cases compared to controls, although there was no independent association found with any of these variables. As regards purpura, arthritis, peripheral neuropathy, anaemia, decrease in C3 and C4, anti-La, and cryoglobulinemia were statistically more frequent compared to controls. Only the decrease in C4, and the presence of cryoglobulins were independently associated with the presence of purpura. Conclusion: Extra-glandular cutaneous manifestations were observed in 14% of the patients. Purpura was the most frequent cutaneous manifestation. This was independently associated with decreased C4 and the presence of cryoglobulins.

Manifestaciones cutáneas en pacientes hemodializados crónicos: Estudio prospectivo sore 100 casos / Cutaneous manifestations in chronic hemodialysis patients: Study with 100 cases

Las manifestaciones cutáneas, son comunes y diversas en los pacientes con insuficiencia renal crónica (IRC), sobre todo, en aquellos en hemodiálisis (HD). Estas pueden ser anteriores al inicio de la diálisis o podrían ser precipitadas por ella. Materiales y Métodos: estudio observacional, descriptivo, de serie de casos, prospectivo a través del examen de piel realizado por dermatólogos, a 100 pacientes con IRC en HD del Servicio de Nefrología del Hospital Provincial del Centenario de Rosario, Argentina durante el período comprendido entre el 1 de Junio de 2013 hasta el 1 de Junio de 2014. Resultados: en este estudio se incluyeron 100 pacientes con IRC en HD, de los cuales 91 presentaron manifestaciones cutáneas. Discusión: la frecuencia de patologías cutáneas en pacientes con IRC en HD, varía del 50 al 100% y generalmente, se observan más de una manifestación cutánea. Esto coincide con lo hallado en nuestro trabajo, donde de los 100 pacientes: 91 presentaban lesiones y de éstos, la mayoría presentaba 2 o más. Respecto de la edad, el rango es amplio y en nuestro trabajo se observó un predominio en el sexo masculino, al igual que en otros estudios.

Skin manifestations are common and diverse in patients with chronic renal failure (CRF), especially those on hemodialysis (HD). These may be prior to initiation of dialysis or could be precipitated by it. Materials and Methods: observational, descriptive, case series, prospective through the skin examination by dermatologists of 100 patients with CKD on HD in the Nephrology Service of Hospital Provincial del Centenario of Rosario, Argentina during the period June 1, 2013 to June 1, 2014. Results: in this study of 100 patients with CRF in HD, of which 91 had cutaneous manifestations were included. Discussion: the frequency of skin diseases in patients with CRF in HD, varies from 50 to 100% and it is usually seen more than one cutaneous manifestation. This coincides with the findings in our study where from 100 patients, 91 had injuries and of these the majority had 2 or more. Regarding age, the range is wide and our study found a predominance in males, as in other studies.

Pérdida del dactilograma durante la senilidad / Loss of the dactylogram during senility

RESUMEN: El dactilograma es propio de la piel humana y se observa en la cara volar de dedos, palmas de manos y plantas de pies, caracterizándose por poseer un ordenamiento único y distinguible. Este patrón es fundamental en identificación humana, por ser único, particular, inmutable, perenne, fácil de observar, comparar y almacenar. Se han descrito cambios en la piel senil, pero pocos han observado desvanecimiento de este patrón. Nuestro objetivo fue evidenciar la pérdida del dactilograma en individuos seniles chilenos y determinar si edad y sexo son variables relacionadas con este fenómeno. La muestra abarcó 487 individuos (120 hombres y 367 mujeres) entre 55 y 107 años. En ellos se registró la presencia o ausencia de dactilograma de cara palmar de pulgar. Los datos obtenidos se procesaron estadísticamente. Se determinó que la edad es una variable relacionada con la pérdida del dactilograma y que a mayor edad, mayor probabilidad de que esta característica no esté presente (a los 82 años, el 25 % no presenta dactilograma, a los 88 años aumenta al 50 % y a los 95 años el 75 % ya no presenta dicho patrón). Así mismo, el sexo determinó diferencias importantes en el desvanecimiento del dactilograma, siendo las mujeres más propensas a presentar pérdida de este patrón (51,77 % de la muestra) que los hombres (37,5 % de la muestra). Pero estos últimos presentan un 42,4 % adicional de riesgo de perder el dactilograma antes que las mujeres. Este trabajo demuestra que el dactilograma se pierde en la senilidad. Hasta ahora este hecho no había sido demostrado. Razón por la cual, este estudio es un aporte significativo en el campo de la morfología y la identificación humana, poniendo de manifiesto que una parte importante de la población chilena ha perdido su identidad basada en las huellas dactilares.

SUMMARY: The dactylogram is of one´s own human skin and can be observed in the volar face of fingers, palms of hands and soles of feet, it is characterized by having a unique and distinguishable order. This pattern is fundamental in human identification because it is unique, particular, immutable, perennial, easy to observe, compare and store. Changes in senile skin have been described, but few have observed the loss of this pattern. Our objective was to evidence the loss of the dactylogram in Chilean senile individuals and establish if age and sex are variables related to this phenomenon. The sample included 487 individuals (120 men and 367 women) between the ages of 55 to 107 years. In them the presence or absence of dactylogram on the palmar face of the thumbs was recorded. The obtained data were processed statistically. It was determined that age is a variable related to the loss of the dactylogram and that the older the subject, the more likely this feature is not present (At 82 years, 25 % do not present dactylogram, at 88 years it increases to 50 % and at 95 years, 75 % no longer have such pattern). Likewise, sex determined significant differences in the dactylogram fading, with women being more prone to the loss of this pattern (51.77 % of the sample) than men (37.5 % of the sample). But the latter have an additional 42.4 % risk of losing the dactylogram earlier than women. This work demonstrates that the dactylogram is lost in senility. So far, this fact had not been proven. Therefore, this study is a significant contribution in the field of morphology and human identification, revealing that an important part of the Chilean population has lost its fingerprint-based identity.

Chikungunya infection in infants / Infecção por Chikungunya em lactentes

Abstract Introduction: the infection of chikungunya virus presents clinical manifestations variables, particularly in infants in which may present multiple cutaneous manifestations. Description: a case series study was carried out in an analytical character of 14 infants (>28 days to < 2 years old) admitted in a hospital between November 2015 and January 2016 with suspected case of chikungunya, by a specific IgM reactive serology. Patients positive for dengue fever, Zika virus, bacterial infections and other exanthematic diseases were excluded. Fever and cutaneous alterations were the most frequent clinical manifestations in 100% of the cases, followed by irritability (64.3%), vomits and arthralgia/arthritis in 35.7% each. Three children presented alterations in the cerebrospinal fluid compatible to meningitis. Anemia frequency was 85.7%. The median white blood cells count was 7.700/mm3 (2.600 to 20.300/mm3). High levels of aminotransferases were observed in three cases (230 to 450 U/L). Antibiotic therapy was indicated in 64.3% of the cases. Two infants needed opioid derivatives for analgesia while others took acetaminophen and/or dipyrone. Discussion: the study shows evident multi-systemic involvement of chikungunya infection in infants. The treatment is supportive, giving special attention to hydration, analgesia, skin care, and rational use of antibiotic therapy.

Resumo Introdução: a infecção pelo vírus chikungunya apresenta manifestações clínicas variáveis, particularmente em lactentes, nos quais parece haver uma multiplicidade de manifestações cutâneas. Descrição: foi feito um estudo tipo de série de casos de caráter analítico com 14 lactentes (>28 dias a < 2 anos) internados entre novembro de 2015 e janeiro de 2016 como caso suspeito de infecção por chikungunya com sorologia IgM específica reagente. Excluídos pacientes com exame positivo para dengue, Zika vírus, infecção bacteriana e outras doenças exantemáticas. As manifestações clínicas mais frequentes foram febre e alterações cutâneas em 100% dos casos, irritabilidade (64,3%), vômitos e artralgia/artrite em 35,7% cada. Três crianças apresentaram alterações liquóricas compatíveis com meningite. A frequência de anemia foi de 85,7%. A mediana de leucometria foi de 7.700/mm3 (2.600 a 20.300/mm3). Níveis aumentados de aminotransferases foram observados em três casos (230 a 450 U/L). Antibioticoterapia foi indicada em 64,3% dos casos. Dois lactentes necessitaram de derivados de opioides para analgesia fixa, enquanto os demais, paracetamol e/ou dipirona. Discussão: o estudo evidencia envolvimento multisistêmico da infecção por chikungunya em lactentes. O tratamento permanece de suporte, com atenção a hidratação, analgesia, cuidados com a pele e ao uso racional de antibioticoterapia.

Tuberous Sclerosis with Severe Cutaneous Manifestation and Multiples Facial Angiofibromas.

Tuberous sclerosis is an extremely variable disease that can affect virtually any organ in the body. The most common findings are cutaneous manifestations, that are critical features in helping to establish diagnosis. We present a case of young man with diagnosis of tuberous sclerosis presenting multiple shagreen patches around the trunk, in the neck and genital region; large plaques with uneven surfaces on the right side of the lower back; and multiple papular lesions in his face, particularly around the nasolabial region, eyebrows and forehead. Considering that tuberous sclerosis is a disease with a highly variable clinical presentation, thus dentists and doctors should be aware of the different manifestations that may be found.

Typical evanescent and atypical persistent polymorphic cutaneous rash in an adult Brazilian with Still's disease: a case report and review of the literature.

Adult onset Still's disease (AOSD) is a systemic auto-inflammatory condition of unknown etiology, characterized by high fever, an evanescent, salmon-pink maculopapular skin rash, arthralgia or arthritis and leukocytosis. AOSD can also present with atypical cutaneous manifestations, such as persistent pruritic coalescent papules or plaques and linear lesions that have highly distinctive pathological features and are usually associated with severe disease. Herein, we present a 31-year-old Brazilian man with both typical Still's rash and atypical persistent polymorphic cutaneous manifestations associated with severe systemic inflammatory response syndrome. Eosinophils that are consistently lacking in the AOSD-associated skin lesions were evident in the skin biopsy of the persistent atypical cutaneous manifestations and were either drug-related or AOSD-associated.