[Algoritmo de tratamiento de la dermatitis atópica en Perú. Consenso de expertos].

Objective: Atopic dermatitis is a chronic, systemic, relapsing disease with dermatological manifestations, which imposes a high burden on patients, families and the health care system and has a high psychological, social, and economic impact and on the quality of life of patients. It mainly affects the pediatric population and, to a lesser extent, the adult population. The clinical presentation varies according to the age and evolution of the disease, and currently there are multiple pharmacological and non-pharmacological therapies available for the symptomatic management of patients. Methods: To present an algorithm for the management of atopic dermatitis, proposed as a series of recommendations on the management, diagnosis, education, and follow-up of these patients. Results: A consensus was reached using the nominal group technique. The methodology was developed in 7 phases, including: posing the research questions, literature search, an initial proposal of recommendations, elaboration of the final recommendations and the management algorithm with three voting cycles, consensus was established with 80% favorability. Conclusions: The result of the consensus process is a management algorithm for patients with mild, moderate/severe atopic dermatitis derived from expert recommendations. The algorithm establishes diagnostic and treatment criteria and provides updated recommendations, including all therapeutic alternatives available in Peru for the management of patients with mild, moderate, and severe atopic dermatitis.

Objetivo: La dermatitis atópica es una enfermedad crónica, sistémica, reincidente, con manifestaciones dermatológicas, que impone una alta carga a los pacientes, las familias y los sistemas de salud, y tiene repercusión psicológica, social y económica, y en la calidad de vida de los pacientes. Afecta principalmente a la población pediátrica y con menor frecuencia a la adulta. Las manifestaciones clínicas varían según la edad y evolución de la enfermedad, y en la actualidad se dispone de múltiples opciones farmacológicas y no farmacológicas para el tratamiento de los pacientes. Métodos: Presentar un algoritmo de tratamiento de la dermatitis atópica, propuesto con una serie de recomendaciones acerca del diagnóstico, tratamiento y seguimiento de los pacientes. Resultados: Se llevó a cabo un consenso de expertos, utilizando la técnica del grupo nominal. La metodología se desarrolló en 7 fases que incluyeron: planteamiento de las preguntas de investigación, búsqueda de la bibliografía, propuesta inicial de las recomendaciones, elaboración de las recomendaciones finales y del algoritmo de tratamiento con tres ciclos de votación. Se estableció el consenso con un 80% de favorabilidad. Conclusiones: El resultado del consenso fue un algoritmo de tratamiento de pacientes con dermatitis atópica leve, moderada-grave, derivado de las recomendaciones de expertos. En el algoritmo se establecen criterios diagnósticos y de tratamiento, y se aportan recomendaciones actualizadas que incluyen las alternativas disponibles en Perú.

Clinical evaluation of infantile hemangiomas treated with atenolol.

Currently, propranolol, is the first line treatment for problematic infantile hemangioma (IH) management. However, serious side effects have been reported. For that reason, atenolol, a hydrophilic selective beta-1 blocker with the potential for fewer side effects, has been explored. A descriptive, observational case series study of 30 patients between the ages one to 5 months with superficial, deep, or mixed IH was conducted between January 2016 and December 2017. Oral atenolol was administered using a single once daily dose of 1mg/kg, which was adjusted for weight gain each month. The IH was assessed using the Hemangioma Activity Score (HAS) at initiation of treatment, four months, and 9 months of age and improvement percentage was calculated at four and nine months of age. A total of 25 patients completed three evaluations. The baseline, four-month, and 9-month HAS were 4.6, 2.39, and 0.65, respectively. Mean improvement percentage at four months of age was 46.76% and at 9 months of age was 85.65%. No side effects were reported. This study suggests atenolol as an effective treatment for IH in almost all cases, especially in patients who initiated treatment before three months of age. It was well tolerated in all our cases.

Dermoscopic findings in neonatal leukemia cutis.

Leukemia cutis is the direct infiltration of cutaneous tissues by leukemic cells and can present as a blueberry muffin baby. We present a case of neonatal leukemia cutis highlighting its dermoscopic features, the presence of fine telangiectatic arborizing vessels distributed within a pink background within all lesions.

Síndrome PHACES: comunicación de un caso con compromiso hepático e intestinal / PHACES syndrome: a report of a case with liver and intestinal involvement

El síndrome PHACES representa un espectro de anormalidades: malformaciones de la fosa posterior, hemangiomas segmentarios, anomalías vasculares, cardíacas, oculares y hendidura esternal o rafe supraumbilical, asociado también a hemangiomatosis extracutánea.Comunicamos el caso de una paciente de 4 meses de edad, con un hemangioma segmentario gigante en la hemicara izquierda, además de la presencia de una malformación de Dandy-Walker, malformación cardiovascular, hendidura esternal e hipotiroidismo.También tenía un hemangioma en el hígado y otras lesiones vasculares en el intestino.

PHACES syndrome represents a spectrum of anomalies such as posterior fossa malformations, segmental hemangiomas, vascular and cardiac abnormalities, eye abnormalities and sternal cleft or raphe supraumbilical, hemangiomatosis extracutánea. We present a 4 months of age female, who presented a segmental giant hemangioma in the left hemiface with Dandy-Walker malformation, cardiovascular malformation, sternal cleft and hypothyroidism, who also presented an hemangioma in the liver and other vascular lesions in the intestine.

Congenital Degos Disease: Case Report and Dermoscopic Findings.

Pediatric Degos disease is rare, with only 36 cases reported in the medical literature. Classically the diagnosis has been established according to pathognomonic histopathologic findings, but when these features are not present, there may be a delay in diagnosis. We report the second congenital case of Degos disease, highlighting the clinical and dermoscopic findings.

Vegetating Candidiasis: A Mimicker of Squamous Cell Carcinoma in Keratitis Ichthyosis Deafness Syndrome.

Keratitis ichthyosis deafness (KID) syndrome is a rare genodermatosis with a high risk of cutaneous malignancy and infections. Infections can induce pseudocarcinomatous epidermal hyperplasia, leading to erroneous diagnosis of squamous cell carcinoma. We present a pediatric case of KID syndrome with vegetating plantar and acral candidiasis and highlight the importance of correct biopsy technique and clinicopathologic correlation in appropriate management.

A randomized, controlled trial of oral propranolol in infantile hemangioma.

BACKGROUND: Oral propranolol has been used to treat complicated infantile hemangiomas, although data from randomized, controlled trials to inform its use are limited. METHODS: We performed a multicenter, randomized, double-blind, adaptive, phase 2-3 trial assessing the efficacy and safety of a pediatric-specific oral propranolol solution in infants 1 to 5 months of age with proliferating infantile hemangioma requiring systemic therapy. Infants were randomly assigned to receive placebo or one of four propranolol regimens (1 or 3 mg of propranolol base per kilogram of body weight per day for 3 or 6 months). A preplanned interim analysis was conducted to identify the regimen to study for the final efficacy analysis. The primary end point was success (complete or nearly complete resolution of the target hemangioma) or failure of trial treatment at week 24, as assessed by independent, centralized, blinded evaluations of standardized photographs. RESULTS: Of 460 infants who underwent randomization, 456 received treatment. On the basis of an interim analysis of the first 188 patients who completed 24 weeks of trial treatment, the regimen of 3 mg of propranolol per kilogram per day for 6 months was selected for the final efficacy analysis. The frequency of successful treatment was higher with this regimen than with placebo (60% vs. 4%, P<0.001). A total of 88% of patients who received the selected propranolol regimen showed improvement by week 5, versus 5% of patients who received placebo. A total of 10% of patients in whom treatment with propranolol was successful required systemic retreatment during follow-up. Known adverse events associated with propranolol (hypoglycemia, hypotension, bradycardia, and bronchospasm) occurred infrequently, with no significant difference in frequency between the placebo group and the groups receiving propranolol. CONCLUSIONS: This trial showed that propranolol was effective at a dose of 3 mg per kilogram per day for 6 months in the treatment of infantile hemangioma. (Funded by Pierre Fabre Dermatologie; ClinicalTrials.gov number, NCT01056341.).

Presentación inusual de morfea en placas en un niño / Unusual presentation of morphea in plates in child

La morfea de presentación infantil se caracteriza por manifestarse como lesiones lineares, localizadas con mayor frecuencia en uno de los miembros o en la región facial. La morfea tiene mayor prevalencia en el sexo femenino. Se presenta el caso de un niño de once años sin morbilidad adicional, con lesiones planas, hiperpigmentadas y atróficas, localizadas en la parte medialy lateral del tercio inferior de ambas piernas. Las lesiones fueron confirmadas histopatologicamente como morfea superficial.

Child onset morphea is usually characterized by linear lesions, more frequently located in one limb or in the facial region, more prevalent in white female children. We report the case of a 11year-old male child without additional morbidity, with atrophic and hyperpigmented flat plaque lesions, located in the inner and outer side of the lower third part of both legs. The lesions were pathologically confirmed as superficial morphea.

Variante ampollar de síndrome de Sweet: presentación de un caso y revisión de la literatura / Bullous Variant of Sweet's syndrome: case report and literature review

El síndrome de Sweet, también llamado dermatosis neutrofílica febril aguda es una enfermedad sistémica de etiología desconocida, poco frecuente en la población general y raramente diagnosticada en la infancia. Se caracterizada por presentar pápulas y/o nódulos eritematovioláceos que coalescen formando placas dolorosas, fiebre y leucocitosis con neutrofilia, y la presencia de un denso infiltrado dérmico neutrofílico en la histopatología cutánea. Presentamos el caso inusual de un adolescente de 13 años de edad que presentó una variante ampollar del síndrome de Sweet.

Sweet’s syndrome or acute febrile neutrophilic dermatosis is a systemic disease of unknown aetiology, very infrequent in general population and rarely diagnosed in childhood. It is characterized by the presence of papules and/or nodules that coalesce to form painful erythematous and violaceous plaques, fever, neutrophilic leucocytosis and a massive neutrophilic dermal infiltrate on skin histopathology. We present the unusual case of a 13 years old adolescent who presented a bullous variant of Sweet syndrome.

Beta-bloqueadores y hemangiomas: de lo fortuito a lo accidental / Beta-blockers and hemangiomas, as incidental to the accidental

Los hemangiomas infantiles son tumores vasculares benignos y los más comunes en la infancia. Se localizan, en un 80% de los casos, en cabeza y cuello. Complicaciones serias ocurren en el 20% de ellos. Los corticoides orales han sido el tratamiento de primera línea para los hemangiomas agresivos y complicados, pero con una respuesta satisfactoria en el 75% de casos, asociada a una carga alta de efectos adversos. Los beta-bloqueadores orales, como el propranolol, son la alternativa actual para estos casos. Ésta droga es actualmente considerada de primera línea dado que ofrece una alta y rápida efectividad sin efectos adversos de riesgo. El timolol tópico se convierte en la mejor alternativa para el tratamiento de los hemangiomas pequeños y localizados.

Infantile hemangiomas are common and benign vascular tumors. In 80% of cases they are located on the head and neck. Serious complications occur in 20% of them. Oral corticosteroids have been the first line treatment in aggressive and complicated hemangiomas, but satisfactory response occurs in 75% of cases associated with a high burden of adverse effects. Oral beta-blockers such as propranolol are the current alternative for these cases. Propranolol is now considered as a first line treatment because it offers high and fast efficiency without serious adverse effects. Topical timolol becomes the best alternative for the treatment of small and localized hemangiomas.

Dermatitis infectiva: una revisión: a propósito de una experiencia peruana / Infective dermatitis: a review: about Peruvian experience

La dermatitis infectiva es una enfermedad eccematosa crónica de la niñez, que siempre compromete el cuero cabelludo y puede progresar a leucemia/linfoma de células T o a paraparesia espástica tropical. Es una condición dermatológica especial que está relacionada a la infección por el retrovirus linfotrópico humano a células T de tipo 1 (HTVL-1). En la niñez la forma de trasmisión más importante es a través de la lactancia materna. La expresión clínica así como su progresión están relacionadas con la carga viral, condiciones inmunológicas del paciente (infestación por Strongiloides stercoralis) y la intensidad de la respuesta inflamatoria. En esta revisión se destacan las características clínicas de esta entidad y se resaltan además sus hallazgos histopatológicos.

Infective dermatitis is a chronic, eczematous dermatitis of childhood that always involves the scalp and may progress to adult T-cell leukemia/lymphoma or tropical spastic paraparesis. It is a special dermatologic condition that has been linked to human T-cell lymphotropic virus type 1 (HTLV-1) infection. The most important route of transmission is vertical through breast-feeding. The clinical expression as well as its progression is related to viral load, immune status of patients (infestation by Strongyloides stercoralis) and the intensity of the inflammatory response. This review highlights the clinical features of this entity and also emphasizes its histopathological findings.

Escrofuloderma asociada a osteítis tuberculosa / Scrofuloderma associated with tuberculous osteitis

La escrofuloderma es una forma de tuberculosis cutánea que resulta de la extensión directa a la piel de un foco tuberculoso contiguo (linfático u óseo), con frecuente fistulización y evolución crónica, pudiendo ocurrir curación espontánea que deja cicatriz retráctil. Se presenta el caso de un paciente varón de 7 años de edad, que presenta dos úlceras en la pared torácica izquierda con un aumento del volumen torácico del mismo lado de 7 meses de evolución; recibe tratamientos antibióticos múltiples y curaciones sin mejoría. En la radiografía de tórax se evidencia compromiso de pleura y arcos costales izquierdos, que es compatible con osteítis tuberculosa. Se realiza biopsia de piel, observándose granulomas tuberculoides con necrosis caseosa y coloración Ziehl Neelsen positiva para BAAR. Inicia tratamiento con tuberculostáticos obteniendo una mejoría clínica evidente. A las 8 semanas de iniciado el tratamiento, se obtiene cultivo positivo para Mycobacterium tuberculosis.

The scrofuloderma is a form of cutaneous tuberculosis that results of the extension of a contiguous tuberculous focus (lymphatic or bone) to the skin, with frequent fistulization and chronic evolution; spontaneous healing may occur in some cases leaving a scar. We present the case of a 7 years old male patient who complained of two ulcers with deformation of the thoracic wall that started 7 months previous to the diagnosis. He received multiple antibiotic treatments without improvement. The chest radiograph showed left pleura and costal arches involvement. Skin biopsy showed the presence of tuberculoid granulomas with caseous necrosis, and Ziehl Neelsen staining was positive for BAAR. The patient received tuberculostatic treatment and had clinical improvement. After 8 weeks of treatment, the culture resulted positive for Mycobacterium tuberculosis.

Granuloma tricofítico de Majocchi: comunicación de un caso / Majocchi trichophytic Granuloma: case report

El granuloma tricofítico de Majocchi es una infección causada por hongos dermatofitos que invaden dermis profunda y tejido celular subcutáneo. Existen pocos reportes de esta entidad. Se han descripto diferentes características clínicas de acuerdo al estado inmune del paciente. Presentamos el caso de una paciente de 8 años de edad con múltiples nódulos ligeramente dolorosos, en cuero cabelludo y cara, de 6 meses de evolución, que había sido tratada con corticoides tópicos y sistémicos. El estudio anatomopatológico evidenció abundantes estructuras micóticas con formación de granulomas en dermis profunda. Se estableció el diagnóstico de granuloma tricofítico de Majocchi y se inició terapia antimicótica con itraconazol, 5 mg/kg/día, con mejoría clínica parcial a la cuarta semana de tratamiento

Trichophytic Majocchi granuloma is an infection by dermatophytes which invade deep dermis and fat. There are few reports about this disease. Some clinical features have been described depending on immune state of patients. We present a case in an 8 years old girl with tenderness nodules in scalp and face of 6 months of evolution, who had been previously treated with topical and systemic steroids. Histological findings showed many fungal structures and a dermal granulomatous inflammatory infiltrate in deep dermis. Diagnosis of trichophytic Majocchi granuloma was done and initiated anti fungal therapy with itraconazole 5 mg/kg once a day with partial improvement at the fourth week of treatment

Púrpura de Henoch-Schõnlein: epidemiología, clínica y seguimiento por cinco años en pacientes hospitalizados en el Instituto Especializado de Salud del Niño, 1995-1999 / Henoch-Schõnlein Purpura: epidemiology, clinical and follow-up for five years in patients hospitalized in Specialized Institute of Child Health, 1995-1999

Objetivo: Determinar las características epidemiológicas, clínicas y el pronóstico de la púrpura de Henoch-Schönlein (PHS) en pacientes hospitalizados en el Instituto Especializado de Salud del Niño (IESN), durante el periodo 1995-1999.Material y métodos: Estudio transversal retrospectivo. Se seleccionaron las historias de pacientes entre 2 y 17 años, hospitalizados con diagnóstico de PHS, que cumplieran con los criterios. Se excluía a todo paciente que hubiera presentado patologías previas con vasculitis o durante el curso de su enfermedad. Los datos obtenidos fueron procesados por SPSS versión 13 y Microsoft Excel 2003. Resultados: Se estudiaron 49 niños, 71% mujeres y 29% varones, el rango de edades fue de 2 a 16 años (media 6.2±3.3), el grupo etario más afectado fue el escolar (63%), seguido del preescolar (33%). Se identificó como posible factor desencadenante la infección de vías respiratorias altas en 14 pacientes (29%), de los cuales sólo uno registró ingesta previa de fármacos. El número de casos de PHS fue mayor en invierno con un 43%. Se observó compromiso cutáneo (100%), articular (63%), digestivo (53%) y renal (20%). El signo de púrpura palpable se encontróen todos los pacientes y los miembros inferiores fueron los más afectados (98%). El patrón articular más frecuente fue el oligoarticular. A nivel gastrointestinal el dolor abdominal fue el signo de mayor presentación (49%), y la afección renal se presentó en un 20%, más frecuentemente con microhematuria. No existió uniformidad en la solicitud de los exámenes auxiliares de laboratorio. Se realizó biopsia de piel en nueve pacientes, encontrándose vasculitis leucocitoclástica en seis de ellos. La inmunofluorescencia directa, realizada en cinco pacientes, fue negativa. Se registraron controles posteriores al alta en solo el 31% de pacientes, de los cuales el 8% registró recurrencia...

Objectives: To determine the epidemiological and clinical characteristics as well as the prognosis of Henoch-Schönlein Purpura (HSP) inhospitalized patients at the Specialized Institute of Child Health (IESN) during the period 1995-1999. Material and Methods: A retrospective cross study. We selected clinical records from patients aged 2 to 17 years, hospitalized al the IESN with a diagnosis of HSP, who met the criteria. All patients who had vasculitis diseases prior or during the course of their illness were excluded. The data was processed by SPSS 13th version and Microsoft Excel 2003. Results: We studied 49 children, 71% women and 29% male, age range was from 2 to 16 years (average 6.2±3.3 years), the age group most affected was the schoolar (63%), followed by preschoolar (33%). The upper respiratory tract infection was identified as a possible trigger in 14 patients (29%), of which only 1 recorded previous ingestion of drugs. The number of cases of HSP was higher in winter with 43%. It is characterized by the association of skin(100%), joint (63%), gastrointestinal (53%) and renal (20%) manifestations. The sign of palpable purpura was found in allpatients and lower limbs were the most affected (98%). The most frequent pattern was the oligoarticular. Abdominal pain was the greatest sign ofsubmission (49%) between the gastrointestinal manifestations, and renal disease was presented by 20%, more often with microscopic hematuria.There was no uniformity in the application of laboratory tests. Skin biopsy was performed in 9 patients, found leukocytoclastic vasculitis in sixof them. The direct immunofluorescence, in 5 patients, was negative. Only 31% of patients were follow up, of whom 8% recorded recurrence. Conclusions: The HSP is characterized by skin, joints, gastrointestinal and renal manifestations. The palpable purpura is the most important sing and constitute the basis of clinical sine qua non to suspect or establish the diagnosis...

Estudio de casos de urticaria anular aguda en pacientes hospitalizados en el servicio de Dermatología del Instituto de Salud del Niño durante el período 2003 al 2004 / Acute annular urticaria case study in patients hospitalized in the service of Dermatology of the Institute of Health of the Child during the period 2003 to 2004

La urticaria aguda en niños es una entidad frecuente, sin embargo su incidencia no está bien establecida. Una variante es la urticaria anular aguda o gigante que ha sido caracterizada en varios trabajos. Objetivo: Caracterizar la urticaria anular aguda en el Instituto Nacional de Salud del Niño. Material y métodos: Estudio observacional no concurrente de serie de casos. Se revisaron historias clínicas de pacientes hospitalizados del servicio de Dermatología del Instituto Nacional de Salud del Niño, Lima-Perú, con criterios de inclusión y exclusión. Resultados:Se hallaron 27 niños con diagnóstico de urticaria anular aguda,las lesiones más frecuentes fueron las placas violáceas y manchas residuales. El área más afectada fue el tórax y dentro de los factores desencadenantes más importantes se encontró el uso de furazolidona en el 59% de los casos. Conclusiones: La urticaria anular aguda o gigante es una reacción de hipersensibilidad con características propias asociada en un alto porcentaje al uso de furazolidona (AU)

Acute urticaria in children is a frequent disorder; however its incidence has not been established. Annular acute urticaria or giant urticaria is a variant which has been reported in several surveys. Objetive: Characterize acute annular urticaria at Instituto Nacional de Salud del Niño, Lima-Peru. Methods: Observational non-concurrent series of cases. We reviewed the medical records of hospitalized patients at Dermatology Service, Instituto Nacional de Salud del Niño, with inclusion and exclusion criteria. Results: We found 27 children with diagnosis of acute annular urticaria; the most frequent lesions were lilaceous plaques and residual patches.The most frequently affected area was chest and the most important triggering factor was the use of furazolidone. Conclusions: Acute annular urticaria is a hypersensitivity reaction with special characteristics, which is associated in a high percentage to use of furazolidone (AU)