Tumor risk markers in recurrent respiratory papillomatosis

Abstract Objectives: This study aims to investigate the pattern of immunoexpression of proteins p16, p53 and Ki-67 in RRP, as well as to evaluate its influence on the number of surgeries that patients have undergone to date and to analyze the benefit of immunohistochemistry in this disease. Methods: Clinical-demographic data and tumor samples were obtained from 33 patients with RRP. The expression of proteins p16, p53 and Ki-67 was analyzed by immunohistochemical method. Results: Most patients had already undergone more than one surgery. The p16 marker was negative in 24.2% of the cases, with little positivity in 27.3% of the cases, moderate in 36.4% and intense in 12.1%. The p53 marker was positive in all cases, with little immunoexpression in 39.4% of cases, moderate in 36.4% and intense in 24.2%. The Ki-67 marker showed nuclear positivity in all lesions, although in varying degrees, with a mean proliferative index ±SD (standard deviation) of 51.7±26. Conclusions: The papillomatous lesions had varying degrees of immunoexpression of proteins p16, p53 and Ki-67, but no specific immunohistochemical pattern was observed. It was found, with statistical difference, that the number of surgeries was higher in cases with greater intensity of p53 expression, without correlation with the other markers. The benefit of immunohistochemistry in recurrent respiratory papillomatosis may lie in the prognostic assessment. However, further studies are needed to evaluate the use of this technique for this purpose. Level of evidence:: 4.

Papilomatosis laringea recurrente con compromiso pulmonar: reporte de caso / Recurrent laryngeal papillomatosis with pulmonary involvement: case report

La papilomatosis respiratoria recurrente es una enfermedad neoplásica causada por el virus del papiloma humano y caracterizada por el crecimiento de lesiones proliferativas exofíticas que afectan la mucosa de las vías respiratorias. En su epidemiología se presenta una distribución bimodal, con una forma juvenil en menores de 20 años, más agresiva, con múltiples lesiones papilomatosas y alta frecuencia de recurrencia, en comparación con la forma adulta. El compromiso pulmonar es poco frecuente y su manejo es un desafío. Se presenta el caso de un varón de 13 años con antecedente de papilomatosis laríngea desde los dos años. El paciente mostró dificultad respiratoria y múltiples nódulos estenosantes en laringe y tráquea, y varios quistes pulmonares visualizados en la tomografía de tórax. Se le realizó exéresis de las lesiones papilomatosas y traqueostomía. Recibió dosis única de bevacizumab 400 mg intravenoso y terapias respiratorias con evolución favorable, sin recurrencias en el seguimiento.

Recurrent respiratory papillomatosis is a neoplastic disease caused by the human papillomavirus and characterized by the growth of exophytic proliferative lesions affecting the mucosa of the respiratory tract. This condition has a bimodal age distribution; the juvenile form affects those under 20 years of age, is more aggressive and presents multiple papillomatous lesions and high frequency of recurrence, compared to the adult form. Pulmonary involvement is rare and challenging to treat. We present the case of a 13-year-old male with a history of laryngeal papillomatosis since the age of two years. The patient showed respiratory distress and multiple stenosing nodules in the larynx and trachea, as well as several pulmonary cysts identified on chest CT. The patient underwent excision of the papillomatous lesions and tracheostomy. Then, the patient received a single dose of intravenous bevacizumab 400 mg and respiratory therapies with favorable evolution, without recurrences during follow-up.

Mesenchymal stem cells-derived exosomal miR-653-5p suppresses laryngeal papilloma progression by inhibiting BZW2

Abstract Objectives: Although miR-653-5p has been validated to participate in the progression of multiple types of cancer, the functional role of exosomal miR-653-5p derived from Mesenchymal Stem Cells (MSCs) in Laryngeal Papilloma (LP) has still remained elusive. Hence, this study aimed to investigate the role of MSCs-derived exosomal miR-653-5p in LP. Methods: LP tissues (n = 15) and adjacent normal tissues (n = 10) were collected to examine the expression level of miR-653-5p. The expression level of miR-653-5p in LP cells and normal cells was also detected. Then, miR-653-5p was overexpressed or silenced to explore its effects on the proliferation, migration, invasion, and apoptosis of LP cells. Thereafter, the effects of exosomal miR-653-5p derived from MSCs on LP cell progression and the potential regulatory mechanism of miR-653-5p were assessed. Results: It was revealed that the expression level of miR-653-5p was downregulated in LP tissues and cells. In addition, miR-653-5p suppressed the proliferation, migration, invasion, and apoptosis of LP cells. Exosomes derived from MSCs played a suppressive role in LP development and mediated the transmission of miR-653-5p to LP cells. Further exploration identified Basic leucine Zipper and W2 domains 2 (BZW2) as the target of miR-653-5p. More importantly, the rescue experiments revealed that MSCs-secreted exosomal miR-653-5p efficiently inhibited the aggressive phenotypes of LP cells, which could be significantly reversed by BZW2 overexpression in LP cells. Conclusion: MSCs-derived exosomal miR-653-5p exerted inhibitory effects on LP progression through targeting BZW2, which provided a novel idea for the therapy of LP. Clinical Trial registration number: chictr-ior-17011021.

Papilomatosis confluente y reticulada de Gougerot-Carteaud / Confluent and reticulated papillomatosis of Gougerot-Carteaud

La papilomatosis confluente y reticulada es una dermatosis infrecuente, benigna, de curso crónico y etiopatogenia desconocida. Actualmente, se acepta que se debe a un trastorno de la queratinización. Se caracteriza por máculas y pápulas hiperpigmentadas, que coalescen en el centro y adoptan un patrón reticular en la periferia. Se la puede confundir con otras patologías, como la pitiriasis versicolor y la acantosis nigricans. Con la administración de minociclina por vía oral suelen obtenerse excelentes resultados. Se comunica el caso de un paciente con diagnóstico de papilomatosis confluente y reticulada. Se describen sus características clínicas, criterios diagnósticos y trata-miento instaurado.

Confluent and reticulated papillomatosis is an infrequent, benign dermatosis of chronic course and unknown etiopathogenesis. Currently it is accepted that this dermatosis is due to a keratinization disorder. It is characterized by hyperpigmented macules and papules that coalesce in the center and adopt a reticular pattern in the periphery. It can be confused with other pathologies such as pityriasis versicolor and acanthosis nigricans. Oral minocycline usually shows excellent results. The present work reports a case of a patient with a diagnosis of confluent and retic-ulated papillomatosis, describing its clinical characteristics, diagnostic criteria and established treatment.

Rol del virus papiloma humano en cabeza y cuello

Resumen El virus papiloma humano (VPH) es un virus de ADN circular doble hebra con alrededor de 200 genotipos. Este virus se relaciona a distintas lesiones tumorales que afectan cabeza y cuello, incluyendo lesiones malignas escamosas causadas por genotipos de alto riesgo como VPH-16 y VPH-18. Esta revisión tiene por objetivo determinar el rol del VPH en distintas patologías, haciendo distinción entre lesiones benignas y malignas, y con particular énfasis en aquellas en que se ha demostrado una asociación causal con el virus, tales como papilomatosis laríngea y carcinoma escamoso orofaríngeo. Además, se analizarán los mecanismos moleculares de daño, métodos de detección y de prevención como la vacunación contra el virus. Es necesario conocer la relevancia del VPH en nuestra especialidad, ya que su determinación puede tener implicancias en cuanto al manejo y pronóstico de los pacientes.

Abstract Human papillomavirus (HPV) is a double stranded circular DNA virus with around 200 genotypes. This virus is related to different tumor lesions that affect the head and neck, including malignant squamous lesions caused by high-risk genotypes such as HPV-16 and HPV-18. The objective of this review is to determine the role of HPV in different pathologies, distinguishing between benign and malignant lesions, and with particular emphasis on those in which a causal association with the virus has been demonstrated, such as laryngeal papillomatosis and oropharyngeal squamous carcinoma. In addition, molecular damage mechanisms, detection and prevention methods such as vaccination against the virus will be analyzed. It is necessary to know the relevance of HPV in our specialty, since its determination may have implications in terms of patient management and prognosis.

Profile of oncological diseases in dogs and clinical effectiveness of autovaccine therapy of papillomatosis / Perfil de doenças oncológicas em caninos e eficácia clínica da terapia com autovacina em papilomatose

Papillomatosis is one of the few diseases in which autovaccine therapy is recommended, especially in the case of mass manifestation in animals. It is noted that papillomas are diagnosed in 15.7% of cases in histological diagnosis of oncological diseases. Therefore, this work studied the profile of oncological diseases in dogs and the clinical effectiveness of autovaccine therapy of papillomatosis. The article gives a morphological description of papillomas used as a biomaterial, and also describes the technological methods of manufacturing an autovaccine used to treat animals against papillomatosis. A therapeutic scheme for administering the vaccine was proposed and tested, and the results of the clinical trial are presented, as well as a reflection on some moments of the mechanism of vaccine therapy for papillomatosis.(AU)

A papilomatose é uma das poucas doenças onde se recomenda a terapia com autovacina, principalmente no caso de manifestação em massa em animais. Nota-se que papilomas são diagnosticados em 15,7% dos casos em diagnóstico histológico de doenças oncológicas. Portanto, este trabalho estudou o perfil das doenças oncológicas em cães e a eficácia clínica da terapia com autovacina em papilomatose. O presente artigo fornece descrição morfológica dos papilomas utilizados como biomaterial, além da descrição dos métodos tecnológicos de fabricação de uma autovacina usada para tratar animais contra a papilomatose. Um esquema terapêutico para a administração da vacina foi proposto e testado, e os resultados do ensaio clínico são apresentados, e é feita uma reflexão sobre alguns momentos do mecanismo da terapia de vacina para papilomatose.(AU)

Caracterización clinicoepidemiológica y terapéutica de niños con papilomatosis respiratoria recurrente / Clinical, epidemiological and therapeutical characterization of children with recurrent respiratory papillomatosis

Introducción: La papilomatosis respiratoria recurrente es una enfermedad infrecuente de la vía aérea que afecta a niños y adultos, causada por el papilomavirus. Objetivo: Caracterizar niños con papilomatosis respiratoria recurrente desde los puntos de vista clínico, epidemiológico y terapéutico, así como evaluar su evolución a corto y mediano plazos. Métodos: Se realizó una investigación observacional, longitudinal y prospectiva de 40 niños con papilomatosis respiratoria recurrente, atendidos en el Servicio de Otorrinolaringología del Hospital Infantil Docente Sur Dr. Antonio María Béguez César de Santiago de Cuba, desde enero del 2010 hasta diciembre del 2016. Resultados: Los pacientes incluidos en el estudio representaron 3,2 % del total de los ingresos. Predominó el sexo masculino (60,0 %); el promedio de edad fue de 8,6 ± 4 años. Existió asociación estadística entre la localización anatómica de las lesiones y el sexo masculino; sin embargo, no hubo dicha asociación con la edad. Las localizaciones orofaríngea (50,0 %) y laríngea (42,5 %) fueron las de mayor observancia. El parto transvaginal se asoció con el mayor número de pacientes que presentaron la citada afección (85,0 %) y el tratamiento con exéresis e interferón se asoció a la evolución satisfactoria en 95,8 % de los afectados. Conclusiones: Las características clínicas y epidemiologias de los pacientes con papilomatosis respiratoria recurrente de esta casuística no difieren del contexto epidemiológico mundial, siendo una entidad de observación poco frecuente, pero no rara en los servicios de pediatría.

Introduction n The recurrent respiratory papillomatosis is an uncommon disease of the airways that affects children and adults, caused by the papillomavirus. Objective: To characterize children with recurrent respiratory papillomatosis from the clinical, epidemiologic and therapeutic points of view, as well as to evaluate their clinical course at short and mid terms. Methods: An observational, longitudinal and prospective investigation of 40 children with recurrent respiratory papillomatosis was carried out, they were assisted in the Otolaryngology Service of Dr. Antonio María Béguez Cesar Teaching Southern Children Hospital in Santiago de Cuba, from January, 2010 to December, 2016. Results: The patients included in the study represented 3.2 % of the total of admissions. The male sex prevailed (60.0 %); the average age was 8.6 ± 4 years. Statistical association existed between the anatomical localization of the lesions and the male sex; however, there was no such association with the age. The oropharyngeal (50.0 %) and laryngeal (42.5 %) localizations were those of more observance. The transvaginal childbirth was associated with the higher number of patients that presented the mentioned disorder (85.0 %) and the treatment with exeresis and Interferon was associated with the satisfactory clinical course in 95.8 % of those affected. Conclusions: The clinical and epidemiological characteristics of patients with recurrent respiratory papillomatosis of this case material don't differ from the world epidemiological context, being an unusual observation entity, but not strange in the pediatrics services.

Características clínicas y epidemiológicas de la papilomatosis respiratoria recurrente en pacientes pediátricos / Clinical and epidemiological characteristics of recurrent respiratory papillomatosis in pediatric patients

La papilomatosis respiratoria recurrente (PRR) es la segunda causa más frecuente de disfonía durante la infancia, cuyo agente causal más frecuente es el virus del papiloma humano serotipos 6 y 11. Con el objetivo de analizar las características clínicas y epidemiológicas de la PPR en pacientes pediátricos admitidos en el Servicio Desconcentrado Hospital Pediátrico Dr. Agustín Zubillaga, se realizó un estudio descriptivo transversal de recolección retrospectiva de datos durante el lapso 2011-2016. Entre las características sociodemográficas se encontró una edad promedio para el momento del diagnóstico de 4,4 ± 2,41 años, siendo los más afectados los preescolares (70%) sin predilección por sexo. Como antecedente prenatal, 90% fue producto de parto vaginal y 30% reportaron asma e infección respiratoria baja. Las características clínicas presentes fueron disfonía (90%), disnea (70%) y estridor (60%) y la localización de las lesiones fueron comisura anterior (100%), repliegues vocales (80% derecho y 90% izquierdo) y vestíbulo (40%). Se reportaron 40% de casos de VPH-6 y 10% de VPH-11. El tratamiento fue quirúrgico en el 100% de los casos, de los cuales 50% requirió una segunda poda y 30% tres podas; 10% de los pacientes ameritaron traqueotomía. Este estudio aporta información sobre la importancia de reconocer tempranamente la disfonía persistente, lo que permite disminuir la demora en el diagnóstico de PRR y facilitar un manejo oportuno con menores tasas de recidiva a largo plazo(AU)

Recurrent respiratory papillomatosis (RRP) is the second most frequent cause of dysphonia during childhood; the most frequent causative agent is human papillomavirus serotypes 6 and 11. In order to describe the clinical and epidemiological characteristics of RRP In pediatric admitted to the Servicio Desconcentrado Hospital Pediátrico Dr. Agustín Zubillaga, a cross-sectional descriptive study was conducted during the 2011-2016 period. The average age of diagnosis was 4.4 ± 2.41 years, with the highest prevalence in preschool children (70%) and with no sex predilection. 90% of patients were product of vaginal delivery and 30% reported asthma and low respiratory infection. The clinical features present were dysphonia (90%), dyspnea (70%) and stridor (60%); location of the lesions were anterior commissure (100%), vocal folds (80% right and 90% left) and vestibule (40%). 40% of patients reported HPV-6 and 10% HPV-11. Treatment was surgical in 100% of cases of which 50% required a second pruning and 30% three pruning; 10% needed a tracheotomy. This study provides information on the importance of early recognition of persistent dysphonia allowing to reduce diagnosis delay of RRP and facilitate timely management with lower rates of long-term recurrence(AU)

Síndrome de Goltz o Hipoplasia dérmica focal / Goltz Syndrome or focal dermal hypoplasia

RESUMEN Introducción: El síndrome de Goltz o hipoplasia dérmica focal es una enfermedad genética rara del grupo de las displasias ectodérmicas con un mecanismo de herencia dominante ligado al cromosoma X. Objetivo: Describir las características clínicas del síndrome de Goltz, su diagnóstico y tratamiento. Presentación del caso: Paciente femenina de 4 años de edad diagnosticada con síndrome de Goltz. Se valora en equipo multidisciplinario con las especialidades de genética, cirugía maxilofacial, estomatología, dermatología, oftalmología, ortopedia y el servicio de otorrinolaringología. Conclusiones: El síndrome de Goltz se caracteriza principalmente por afectación cutánea; anomalías oculares, dentales, faciales y esqueléticas; afectación del aparato gastrointestinal, urinario, cardiovascular y sistema nervioso central con grado variable de severidad. Su diagnóstico es clínico. La atención interdisciplinaria es fundamental para el adecuado diagnóstico y tratamiento; su pronóstico depende del grado de afectación(AU)

ABSTRACT Introduction: Goltz syndrome also known as focal dermal hypoplasia is a rare genetic disease in the ectodermal dysplasia´s group and with a mechanism of dominant inheritance linked to the X chromosome. Objectives: To describe the clinical characteristics of the Goltz syndrome, its diagnosis and treatment. Case presentation: Case of a 4 year-old female patient diagnosed with Goltz syndrome. She was studied by a multidisciplinary team including Genetics, Maxillofacial Surgery, Stomatology, Dermatology, Ophthalmology, Orthopedics and ORL specialists. Conclusions: Goltz syndrome or focal dermal hypoplasia is mainly characterized by skin affectations; eyes, dental, skeletal, and face anomalies; gastrointestinal tract, urinary, cardiovascular and central nervous systems´ affections with varying degrees of severity. The diagnosis is clinical. A multidisciplinary approach is essential for a proper diagnosis and treatment; and prognosis depends on the grade of severity(AU)

Papilomatosis traqueobronquial y pulmonar sin compromiso de la laringe tratada con bevacizumab endovenoso en un niño / Tracheobronchial and pulmonary papillomatosis without involvement of the larynx treated with intravenous Bevacizumab in a child

La papilomatosis respiratoria recurrente es una neoplasia benigna infrecuente que, comúnmente, afecta la vía aérea superior con predilección por la laringe. El compromiso traqueobronquial aislado es muy raro. El diagnóstico y el tratamiento de esta enfermedad constituyen un desafío, debido a su manifestación clínica inespecífica y su naturaleza recurrente. Se presenta a un varón de 6 años con diagnóstico de asma refractario al tratamiento, sin historia ni evidencia de papilomatosis laríngea. El examen endoscópico reveló papilomatosis traqueobronquial extensa, y la tomografia computada, compromiso pulmonar. Recibió terapia adyuvante con bevacizumab endovenoso, con muy buena respuesta. Se alerta a los pediatras para considerar esta rara neoplasia traqueobronquial en todo niño con síntomas de asma atípicos o que no mejoran con el tratamiento médico convencional.

Recurrent respiratory papillomatosis is an infrequent benign neoplasm that commonly affects the upper airway with a predilection for the larynx. Isolated tracheobronchial involvement is very rare. Diagnosis and treatment of this disease is a challenge due to its non-specific clinical manifestation and its recurrent nature. We present a 6-year-old male with a diagnosis of asthma refractory to treatment, without history or evidence of laryngeal papillomatosis. The endoscopic examination revealed extensive tracheobronchial papillomatosis and the computed tomography, pulmonary involvement. He received adjuvant therapy with intravenous Bevacizumab with very good response. We alert pediatricians to consider this rare tracheobronchial neoplasm in all children with atypical asthma symptoms or in those who do not improve with conventional medical treatment.

Vacuna contra el virus papiloma humano como tratamiento para la papilomatosis respiratoria recurrente / Human papilloma virus vaccination treatment for recurrent respiratory papillomatosis

RESUMEN Una de las manifestaciones clínicas del virus papiloma humano (VPH) es la papilomatosis respiratoria recurrente (PRR), que se caracteriza por la proliferación de lesiones epiteliales verrucosas recurrentes en la mucosa respiratoria, pudiendo progresar a obstrucción de vía aérea o presentar transformación maligna. El tratamiento de primera línea quirúrgico, pero dada su alta recurrencia ha tomado peso el tratamiento adyuvante, como la vacuna tetravalente contra VPH. Sin embargo, existe controversia respecto a su eficacia. El objetivo de esta revisión es analizar la efectividad de la vacuna contra VPH como tratamiento adyuvante de la PRR, para lo cual hicimos una revisión de la literatura sobre la efectividad de la vacuna tetravalente contra VPH para PRR, realizando una búsqueda en diversas fuentes: Pubmed, MEDLINE, EMBASE, Cochrane, Google Scholar y Epistemonikos. Se seleccionaron los estudios que responden a la pregunta y se analizaron los datos de los estudios primarios. Se encontraron cinco estudios primarios no aleatorizados, todos a favor de la vacuna como tratamiento. Concluimos con baja certeza de evidencia, que la vacuna es posiblemente efectiva para pacientes con PRR en disminuir el número de recurrencias, aumentar el intervalo entre cirugías, lograr remisión completa o parcial de la enfermedad y aumentar significativamente los títulos de anticuerpos anti-VPH.

ABSTRACT A clinical manifestation of human papillomavirus (HPV) is recurrent respiratory papillomatosis (RRP), characterized by the proliferation of recurrent verrucous epithelial lesions in the respiratory mucosa, which may progress to airway obstruction or malignant transformation. First-line treatment is surgery, but given its high recurrence the use of adjuvant therapy, such as the quadrivalent vaccine, has gained importance. However, there is controversy regarding its effectiveness. To analyze the effectiveness of the HPV vaccine as an adjuvant treatment for RRP a review of the literature on the effectiveness of HPV tetravalent vaccine for RRP was performed by searching databases such as Pubmed, MEDLINE, EMBASE, Cochrane, Google Scholar and Epistemonikos. We selected the studies that answered the question and analyzed the data from all of which supported the vaccine as treatment. None were randomized controlled trials. We conclude, with low certainty of evidence, that the vaccine is possibly effective for RRP in decreasing the number of recurrences, increasing the intersurgical interval, achieving complete or partial remission of disease and significantly increasing anti-HPV antibodies.

Juvenile-onset recurrent respiratory papillomatosis with pulmonary involvement and carcinomatous transformation

Papilloma associated with recurrent respiratory papillomatosis (RRP), caused by human papilloma virus (HPV) infection types 6 and 11, is the most common benign neoplasm of the larynx. The clinical features of RRP vary widely from mild to aggressive forms. RRP in children is known as juvenile-onset recurrent respiratory papillomatosis (JORRP). Its outcome may be poor or even fatal due to the high rate of recurrence and eventual spread to the entire respiratory tract. Pulmonary invasion is reported to occur in 3.3% of patients with RRP, and malignant transformation in 0.5% of patients. We report the case of a 39-year-old female patient with a diagnosis of JORRP from the age of 3 years, with extensive bilateral pulmonary involvement and malignant transformation. Analysis of the papilloma and carcinomatous tissues revealed the presence of HPV type 11, which is associated with rapid and aggressive progression. We discussed the case on the basis of a literature review on pulmonary invasion, malignant transformation, and HPV 11 aggressiveness.

Paciente con papilomatosis laringo-traqueo-bronquial recurrente y empiema at necessitates Dificultades de diagnóstico y manejo / Patient with recurrent laryngeal-tracheo-bronchial papillomatosis and empyema at necessitates Difficulties in diagnosis and management

Resumen Paciente masculino de 17 años, con cuadro de PLR desde los 18 meses, al que se le han realizado 85 intervenciones a nivel laringo-traqueal, por lo que requirió traqueostomía permanente desde los cinco años, presentando cuadros de neumonías a repetición con invasión papilomatosa a pulmón de predominio derecho con aislamiento de actinomicetos; desarrolló empiema at neccesitatis del que se aislaron múltiples gérmenes multirresistentes con severo compromiso del parénquima pulmonar por lo que requirió neumonectomía radical derecha en intento de controlar la infección. Fue manejado con anfotericina B deoxicolato, meropenem, tigeciclina y polimixina B a pesar de lo cual presenta empeoramiento del cuadro clínico con posterior choque séptico refractario que lo llevó a la muerte.

Abstract A 17-year-old male patient, with a clinical picture of RRP since18 months of age, who underwent 85 interventions at the laryngo-tracheal level, requiring permanent tracheostomy from the age of 5, presenting recurrent pneumonia with lung papillomatous invasion of right predominance with actinomycete isolation. He developed empyema at neccesitatis from which multiple multiresistant organisms with severe compromise of the pulmonary parenchyma were isolated, which required a right radical pneumonectomy in an attempt to control the infection. He was managed with am-photericin B deoxycholate, meropenem, tigecycline and polymyxin B, despite which he presented worsening of the clinical picture with subsequent refractory septic shock that led to his death.

Pegylated interferon for treating severe recurrent respiratory papillomatosis in a child: case report

ABSTRACT CONTEXT: Recurrent respiratory papillomatosis (RRP) is the most common laryngeal tumor. During childhood, it may present in extremely severe forms defined by the need for frequent surgical procedures to relieve respiratory distress and/or involvement of extralaryngeal sites such as lung involvement. Adjuvant therapies are indicated in these cases and interferon is one of the options. Pegylated interferon is more effective than conventional alpha interferon and, given its reported results in relation to treating hepatitis C over the past decade, we hypothesized that this might be more effective than conventional interferon also for treating respiratory papillomatosis. Use of a treatment strategy that eliminates the need for general anesthesia is particularly appealing, yet obtaining approval for use of medications that are not currently used for this purpose is challenging. CASE REPORT: We report the case of a child with severe RRP that had been followed for the preceding six years, who was treated with pegylated interferon after failure of other adjuvant therapies. There was noticeable improvement in the frequency of surgical procedures, which was regarded very receptively, considering the child's history and previous response to other therapies. CONCLUSION: Pegylated interferon may be a good option for diminishing the need for surgical intervention in severe cases of recurrent respiratory papillomatosis.

Manejo anestésico de paciente pediátrico con obstrucción severa de vía aérea causada por Papilomatosis / Anesthetic management in pediatric patient with severe airway obstruction caused by papilloma virus

La papilomatosis laríngea es una enfermedad de etiología viral que puede ser transmitida en el canal de parto o por la sangre materna. Un niño de 2 años con grado III de Obstrucción Laríngea fue programado para diagnóstico por fibrolaringóscopia. Discutimos el manejo perioperatorio del caso con las diversas estrategias de ventilación. Se sugiere mantener la ventilación espontánea hasta la intubación ya que la ventilación de la máscara facial o laríngea podría ser difícil y la intubación impide el paso del fibrolaringoscopio y la valoración completa de la larínge.

Laryngeal papillomatosis is a viral etiology desease that can be transmitted at birth through vaginal way or maternal blood. A 2-year-old boy with Laryngeal Obstruction III grade was scheduled for fibrolaryngoscopy diagnosis. We discuss the perioperative management of the case with the various ventilation strategies. It is suggested to maintain spontaneous ventilation until intubation, since ventilation with facial or laryngeal mask could be difficult and intubation prevent the passage of the fibrolaryngoscope for a complete laryngeal evaluation.

Papilomatosis laríngea: una causa poco frecuente de disfonía en el niño. Serie de casos / Laryngeal papillomatosis: A rare cause of dysphonia in the child. Case series

La papilomatosis laríngea juvenil es una enfermedad infrecuente causada por el virus del papiloma humano, principalmente, los tipos 6 y 11. Es el tumor laríngeo benigno más común en los niños. Debe pensarse, en esta patología, en todo niño con disfonía persistente y progresiva, acompañada o no de estridor y dificultad respiratoria. La laringoscopía flexible con anestesia local permite visualizar las típicas lesiones de características verrugosas. El diagnóstico se confirma mediante la anatomía patológica. El tratamiento existente es paliativo y consiste, principalmente, en la escisión quirúrgica de los papilomas para mantener la vía aérea sin obstrucción y mejorar la calidad de la voz, pero tiene una alta tasa de recidiva. Se presentan 20 pacientes con papilomatosis laríngea juvenil. Se describen las manifestaciones clínicas, el diagnóstico y el tratamiento de esta patología.

Juvenile laryngeal papillomatosis is an uncommon disease caused by human papilloma virus, mainly types 6 and 11. It is the most common benign laryngeal tumor in children. This condition should be suspected in any children with persistent and progressive dysphonia with or without stridor and respiratory distress. Flexible laryngoscopy under local anesthesia allows to visualize the typical wart-like lesions. The diagnosis is confirmed by pathological anatomy. Existing treatment is palliative and consists mainly of the surgical excision of the papillomas to maintain the airway without obstruction and to improve the quality of the voice, but it has a high rate of relapse. We present 20 patients with juvenile laryngeal papillomatosis. We describe the clinical manifestations, the diagnostic methods and the treatment of this pathology.

Pigmentación macular eruptiva idiopática: presentación de dos casos clínicos y revisión de la literatura / Idiopathic eruptive macular pigmentation: presentation of two clinical cases and review of the literature

La pigmentación macular eruptiva idiopática es una enfermedad rara, descrita por Degos en 1978. Se han reportados 57 casos en la literatura. Afecta principalmente a niños entre 6 a 14 años, se caracteriza por manchas hiperpigmentadas color marrón en tronco, cuello y región proximal de extremidades, que aparecen en forma gradual y se resuelve en un periodo de meses o años. Casos clínicos. Masculino de 7 años de edad y femenino de 12 años de edad, con dermatosis diseminada a cabeza, tronco y extremidades que afecta cara, cuello, tronco en todas sus superficies y la parte proximal de las extremidades, constituida por manchas hiperpigmentadas de color gris-marrón de 0.5 ­ 1.3 cm, límites bien definidos, no confluentes, no pruriginosas, sin tratamiento previo. En las biopsias de piel se observó pigmentación de la capa basal, con melanófagos en dérmis. Conclusión. Los casos clínicos descritos cumplen los criterios de Sanz de Galdeano y cols: a) erupción macular azul pizarra o marrón no confluyente, b) ausencia de enfermedades cutáneas inflamatorias previas, c) sin medicación previa, d) estudio histológico con hiperpigmentación de la capa basal, melanófagos en dermis e infiltrado liquenoide y e) conteo de mastocitos normales. El diagnóstico diferencial se hace con erupción por drogas, hiperpigmentación postinflamatoria, mastocitosis, liquen plano pigmentado y eritema discrómico perstans. Es importante reconocer la enfermedad por su comportamiento autorresolutivo (AU)

Idiopathic eruptive macular pigmentation is a rare disease, described by Degos in 1978. There have been 57 cases reported in the literature. It mainly affects children between 6 and 14 years old, is characterized by hyperpigmented brown macules on the trunk, neck and proximal part of limbs that appear gradually and disappear during a period of several months to years. Clinical cases: A 7-year-old male and a 12-year-old female with disseminated skin lesions in the head, trunk and limbs, involving face, neck, all surfaces of the trunk and proximal parts of the limbs, consisting of hyperpigmented gray-brown well-defined isolated macules measuring 0.5 - 1.3 cm in diameter, non-pruritic, without any treatment. In the skin biopsies, pigmentation of the basal layer is observed, with melanophages in dermis. Conclusion: These cases fulfill the criteria of Sanz de Galdeano et al: a) black blotchy or non-confluent brown macular rash, b) absence of previous inflammatory skin diseases, c) without previous medication, d) histological study with hyperpigmentation of the basal layer, melanophages in the papillary dermis and lichenoid infiltrate, e) normal mast cell count. The differential diagnosis is with drug eruption, postinflammatory hyperpigmentation, mastocytosis, pigmented lichen planus and erythema dyschromicum perstans. It is important to recognize the disease because of its self- resolving behavior (AU)

Cáncer orofaríngeo y virus del papiloma humano en cabeza y cuello: relaciones fisiopatológicas y epidemiológicas / Oropharyngeal carcinoma and papiloma virus in head and neck: phisiopathological and epidemiological relations

Resumen El Virus del Papiloma Humano (VPH) es una enfermedad diagnóstico frecuente, transmitida por contacto entre piel y mucosas. Se han descrito múltiples relaciones etiológicas con neoplasias en órganos del sistema genital y reproductor y a nivel de cabeza y cuello. Éste se ha agrupado y caracterizado mediante análisis filogenéticos y en su secuencia de nucleótidos y aminoácidos. EL VPH progresa por las capas de epitelio de piel o mucosa induciendo malignización celular. El diagnóstico se realiza mediante diversas técnicas de PCR o de hibridación in situ. El carcinoma orofaríngeo es una patología neoplásica con una presentación clínica variada. Su asociación a VPH es inmunohistoquímicamente detectable, y se asocia a una presentación más frecuente en jóvenes, en hombres, con una localización anatómica mejor definida, y con una expresión clínica más florida y temprana. Actualmente se ha documentado un incremento en los casos de carcinoma orofaríngeo a pesar del descenso de su principal factor de riesgo; el tabaquismo. Su incidencia y mortalidad continúan siendo relativamente bajas en comparación con otros tumores en cabeza y cuello. Este incremento se ha asociado etiológicamente a la infección de VPH con subtipos de mayor riesgo de malignidad como el VPH 16 y 18. A nivel nacional existen diferencias estadísticas con respecto a los datos foráneos, sin embargo la cantidad de estudios realizados en nuestra población y los sesgos asociados al subregistro de la enfermedad son un factor importante a tomar en cuenta. Es necesario realizar una mayor cantidad de estudios en la población costarricense para determinar el comportamiento real de esta enfermedad y su relación etiológica con el carcinoma de orofaringe en nuestro país.

Abstract The Human Papilloma Virus (HPV) is a frequently diagnosed sexually transmitted disease, transmitted by contact between skin and mucous membranes. There are multiple etiological relationships between HPV infections and neoplasms in organs of the reproductive and genital system and at head and neck. Those belonging to HPV have been grouped and characterized by phylogenetic analysis and by its sequence of nucleotides and amino acids. HPV progresses through the layers of skin or mucosal epithelium inducing malignant transformation in these cells. The diagnosis of this infection is performed using various techniques of PCR and in situ hybridization. Oropharyngeal carcinoma is a neoplastic disease with a varied clinical presentation. Its association with HPV is detectable by immunohistochemic methods. It's also associated more frequent with young people, men, presentation with a better defined anatomic location and an earlier clinical expression. Currently it has been documented an increase in cases of oropharyngeal carcinoma despite the fall of their main risk factor; smoking. Its incidence and mortality continue to be relatively low in comparison with other head and neck tumors. This increase has etiologically been associated with HPV infection; and with subtypes of increased risk of malignancy such as HPV 16 and 18; as with changes in the behavior of the disease. National statistics differ from the foreign data, however the low amount of studies in our population and the biases associated with underreporting of the disease are an important factor to take into account. It is necessary to perform a greater number of studies in the Costa Rican population to determine the actual behavior of this disease and its etiological relation to carcinoma of the oropharynx.

Clinical and Histopathologic Study of Confluent and Reticulated Papillomatosis by Anatomic Site and Age

BACKGROUND: Confluent and reticulated papillomatosis (CRP) is an uncommon dermatosis characterized by hyperpigmented scaly macules or papillomatous papules coalescing into confluent patches or plaques centrally with a reticular pattern peripherally. Few studies regarding this have been reported in the literature. OBJECTIVE: The purpose of this study was to investigate the clinical and histologic characteristics of CRP in Korean patients according to anatomic site and age. METHODS: We retrospectively reviewed the medical records, clinical photographs, and 40 histopathologic slides of 29 patients diagnosed with CRP. RESULTS: Sixteen adults and 13 adolescents were included. The predominantly involved anatomic sites were the trunk (89.7%) and axillae (27.6%); flexural area involvement was mainly found in the adolescent group. The mean disease duration was 2.3 times longer in the adult group than in the adolescent group. The histopathologic features included hyperkeratosis (95.0%), acanthosis (87.5%), and papillomatosis (77.5%) in the epidermis. In the adult group, only 10 specimens (52.6%) showed mild to moderate papillomatosis. There was no relationship between the histologic findings and disease duration; however, the former was associated with the anatomic site. CONCLUSION: The present study is the first to present the clinical and histologic features of CRP according to age and anatomic site in Korean patients. The patients in this study more frequently had fungal infections (31%) than patients in a previous study (12.8%). Histologic changes were more prominent in the adolescent group than in the adult group and in the flexural area than in the non-flexural area.