Post-infectious pityriasis rubra pilaris in pediatric patients. / Pitiriasis rubra pilaris posinfecciosa en pacientes pediátricos.

Pityriasis rubra pilaris (PRP) is a rare skin condition. The etiology of PRP is unknown; however, it has been associated with infections, autoimmune diseases, and neoplasms. Here we describe the cases of 2 pediatric patients with PRP triggered by a respiratory syncytial virus infection concurrently with obstructive bronchial syndrome. PRP resolved after treatment with topical emollients, topical corticosteroids, and calcineurin inhibitors.

La pitiriasis rubra pilaris (PRP) es una enfermedad dermatológica poco frecuente. Se desconoce su etiología, sin embargo, se ha asociado a infecciones, enfermedades autoinmunes y neoplasias. Se describen los casos de dos pacientes pediátricos que presentaron PRP gatillada por una infección por virus sincicial respiratorio mientras cursaban un síndrome bronquial obstructivo. Los cuadros de PRP remitieron luego del tratamiento tópico con emolientes, corticoesteroides tópicos e inhibidores de la calcineurina.

Xantomas tuberosos en un paciente pediátrico: la clave en el diagnóstico precoz de una patología grave y poco frecuente / Tuberous xanthomas in a pediatric patient: a clue to the early diagnosis of a severe and infrequent disease

Los xantomas cutáneos reflejan el depósito de lípidos en la piel y pueden ser la única manifestación temprana de dislipidemias de inicio en la infancia. Las características y distribución de los xantomas orientan a la patología de base; los xantomas tuberosos tienen una fuerte asociación con la hipercolesterolemia homocigota familiar, una patología muy infrecuente. Su detección temprana otorga una ventana terapéutica para prevenir la ateroesclerosis acelerada y la mortalidad. Se presenta el caso de una paciente que comenzó a los dos años con xantomas tuberosos, que fueron la clave diagnóstica para identificar la hipercolesterolemia homocigota familiar subyacente.

Cutaneous xanthomas reflect lipid deposition on the skin and may be the only early manifestation of a childhoodonset dyslipidemia. Characteristics and distribution of the xanthomas signalize the underlying pathology, tuberousxanthomas being strongly associated with homozygous familial hypercholesterolaemia, an extremely rare condition. Its early detection provides a therapeutic window to prevent accelerated atherosclerosis and mortality. We present the case of a patient who started at two years with tuberous xanthomas, which were the diagnostic clue to identify the underlying homozygous familial hypercholesterolaemia.

[Tuberous xanthomas in a pediatric patient: a clue to the early diagnosis of a severe and infrequent disease]. / Xantomas tuberosos en un paciente pediátrico: la clave en el diagnóstico precoz de una patología grave y poco frecuente.

Cutaneous xanthomas reflect lipid deposition on the skin and may be the only early manifestation of a childhoodonset dyslipidemia. Characteristics and distribution of the xanthomas signalize the underlying pathology, tuberous xanthomas being strongly associated with homozygous familial hypercholesterolaemia, an extremely rare condition. Its early detection provides a therapeutic window to prevent accelerated atherosclerosis and mortality. We present the case of a patient who started at two years with tuberous xanthomas, which were the diagnostic clue to identify the underlying homozygous familial hypercholesterolaemia.

Los xantomas cutáneos reflejan el depósito de lípidos en la piel y pueden ser la única manifestación temprana de dislipidemias de inicio en la infancia. Las características y distribución de los xantomas orientan a la patología de base; los xantomas tuberosos tienen una fuerte asociación con la hipercolesterolemia homocigota familiar, una patología muy infrecuente. Su detección temprana otorga una ventana terapéutica para prevenir la ateroesclerosis acelerada y la mortalidad. Se presenta el caso de una paciente que comenzó a los dos años con xantomas tuberosos, que fueron la clave diagnóstica para identificar la hipercolesterolemia homocigota familiar subyacente.

Síndrome de reacción a drogas con eosinofilia y síntomas sistémicos asociado a herpesvirus humano 6. Caso pediátrico tratado con ciclosporina y corticoides / Drug reaction with eosinophilia and systemic symptoms syndrome associated with human herpesvirus 6. A pediatric case treated with cyclosporine and corticosteroids

La reacción a drogas con eosinofilia y síntomas sistémicos es una reacción adversa cutánea rara, potencialmente grave. Puede presentar fiebre, erupción cutánea polimorfa, edema facial y/o linfoadenopatías. La reactivación del virus herpes humano tipo 6 se asocia a un curso más grave y/o prolongado.Un lactante de 22 meses en tratamiento con fenobarbital presentó lesiones eritematopapulares, fiebre, leucocitosis, proteína C reactiva elevada y alteración de pruebas hepáticas. Se realizó biopsia de piel compatible con reacción adversa a drogas. Se trató con corticoides sistémicos e inmunoglobulina intravenosa sin respuesta. La reacción en cadena de la polimerasa para virus herpes humano tipo 6 resultó positiva. Se inició ciclosporina más prednisona, con buena respuesta. Existe poca evidencia del uso de ciclosporina en adultos, cuando los corticoides sistémicos son inefectivos. Este es el primer reporte pediátrico Podría ser una alternativa efectiva o un complemento de los corticosteroides sistémicos cuando no responde a tratamientos convencionales.

Drug reaction with eosinophilia and systemic symptoms is a rare and potentially serious skin adverse reaction, with fever, polymorphous skin rash, facial edema, and/or lymphadenopathy. Reactivation of human herpes virus type 6 has been associated with a more severe and/or prolonged course. A 22-month-old infant under phenobarbital treatment developed erythematous-papular lesions, fever, leukocytosis, elevated C-reactive protein, and abnormal liver tests. The skin biopsy was compatible with an adverse drug reaction. Treatment with systemic corticosteroids and intravenous immunoglobulin had no response. Polymerase chain reaction for human herpesvirus type 6 was positive, and cyclosporine plus prednisone was started with a good response. There is little evidence for the use of cyclosporine in adults when systemic corticosteroids are ineffective. This is the first report of pediatric drug reaction with eosinophilia and systemic symptoms treated with cyclosporine, which could be an effective alternative or an adjunct to systemic corticosteroid therapy unresponsive to conventional treatments.

[Síndrome de reacción a drogas con eosinofilia y síntomas sistémicos asociado a herpesvirus humano 6. Caso pediátrico tratado con ciclosporina y corticoides]. / Drug reaction with eosinophilia and systemic symptoms syndrome associated with human herpesvirus 6. A pediatric case treated with cyclosporine and corticosteroids.

Drug reaction with eosinophilia and systemic symptoms syndrome associated with human herpesvirus 6. A pediatric case treated with cyclosporine and corticosteroids elevated C-reactive protein, and abnormal liver tests. The skin biopsy was compatible with an adverse drug reaction. Treatment with systemic corticosteroids and intravenous immunoglobulin had no response. Polymerase chain reaction for human herpesvirus type 6 was positive, and cyclosporine plus prednisone was started with a good response. There is little evidence for the use of cyclosporine in adults when systemic corticosteroids are ineffective. This is the first report of pediatric drug reaction with eosinophilia and systemic symptoms treated with cyclosporine, which could be an effective alternative or an adjunct to systemic corticosteroid therapy unresponsive to conventional treatments.

La reacción a drogas con eosinofilia y síntomas sistémicos es una reacción adversa cutánea rara, potencialmente grave. Puede presentar fiebre, erupción cutánea polimorfa, edema facial y/o linfoadenopatías. La reactivación del virus herpes humano tipo 6 se asocia a un curso más grave y/o prolongado. Un lactante de 22 meses en tratamiento con fenobarbital presentó lesiones eritematopapulares, fiebre, leucocitosis, proteína C reactiva elevada y alteración de pruebas hepáticas. Se realizó biopsia de piel compatible con reacción adversa a drogas. Se trató con corticoides sistémicos e inmunoglobulina intravenosa sin respuesta. La reacción en cadena de la polimerasa para virus herpes humano tipo 6 resultó positiva. Se inició ciclosporina más prednisona, con buena respuesta. Existe poca evidencia del uso de ciclosporina en adultos, cuando los corticoides sistémicos son inefectivos. Este es el primer reporte pediátrico Podría ser una alternativa efectiva o un complemento de los corticosteroides sistémicos cuando no responde a tratamientos convencionales.

Reporte de manejo exitoso con simvastatina y ezetimibe en alopecia areata / A report of successful management with simvastatin plus ezetimibe in alopecia areata

La alopecia areata es un tipo común de alopecia no cicatricial. Aunque la patogénesis exacta permanece sin dilucidar, se piensa que la alopecia areata tiene una etiología multifactorial en donde se interrelacionan predisposición genética y factores ambientales. En pacientes susceptibles, se han documentado que el estrés, infecciones y microtraumas disminuyen las citoquinas inmunosupresoras que normalmente mantienen el privilegio inmune del folículo piloso. Actualmente no hay terapia curativa para la alopecia areata, aunque ciertos tratamientos pueden inducir el crecimiento del cabello en un porcentaje de pacientes. Se postula que la simvastatina restablece el privilegio inmune y ezetimibe aportaría un efecto inmunomodulador y antiinflamatorio. Se presenta el caso de una mujer de 23 años con alopecia areata, exitosamente tratada con simvastatina y ezetimibe.

Alopecia areata is a common type of non-scarring alo¬pecia. Although the exact pathogenesis remains elusive, alopecia areata is thought to have a multifactorial etiology described as an interplay of genetic predisposition and environmental exposures. In patients with genetic susceptibility, stress, infection, and microtrauma have been documented to decrease immunosuppressive cytokines that generally maintain the hair follicle's immune privilege. There is currently no curative therapy for alopecia areata, although some treatments can induce hair growth in a percentage of patients. It has been postulated that simvastatin reestablishes the immune privilege, and ezetimibe would provide an immunomodulatory and anti-inflammatory effect. We report a case of a 23 years-old woman with alopecia areata successfully treated with simvastatin/ezetimibe.

Lupus eritematoso asociado a eritema multiforme: Síndrome de Rowell. Reporte del primer caso en Chile

Rowell's syndrome is characterized by an association of lupus erythematosus and erythema multiforme, with distinctive laboratory findings. Its treatment is similar to lupus. We report a 16-year-old female presenting with skin lesions, laboratory and histology consistent with this entity. Because of the spread of cutaneous involvement and development of epidermal detachment, she required therapy with intravenous corticosteroids and gamma globulin.

Incidencia de síndrome de Stevens-Johnson y necrólisis epidérmica tóxica en Chile años 2001-2015 y su asociación con latitud / Hospital discharges due to Stevens-Johnson syndrome and toxic epidermal necrolysis in Chile from 2001 to 2015

Background: Stevens-Johnson Syndrome (SSJ) and Toxic Epidermal Necrolysis (NET) are infrequent and life-threatening mucocutaneous diseases, which occur predominantly as adverse drug reactions. Aim: To describe the frequency of SSJ and NET diagnoses at a national level, estimate their incidence and describe their distribution among the different regions of the country. Material and Methods: Analysis of hospital discharge databases available at the website of the Chilean Ministry of Health searching for the tenth version of the International Classification of Diseases (ICD 10) codes for SSJ or NET, between 2001 and 2015. Results: We analyzed 24,521,796 hospital discharges nationwide. SSJ caused 855 discharges, with a lethality of 2%. NET caused 128 discharges with a lethality of 16%. The global cumulative incidence was 3.87 cases per million inhabitants per year nationwide, with a trend line to increase incidence towards the regions of higher latitude. Conclusions: SSJ and NET are dermatological emergencies with high mortality. The increase in incidence towards regions at higher latitudes may suggest an association between these conditions and lower levels of vitamin D, correlated with latitude and exposure to UV radiation.

Diagnosis of elephantiasis nostras verrucosa as a clinical mani-festation of Kaposi's sarcoma.

Elephantiasis nostras verrucosa, a rare manifestation of Kaposis sarcoma, is a progressive cutaneous hypertrophy caused by chronic non-filarial lymphedema secondary to obstruction of the lymphatic system that can lead to severe disfigurement of parts of the body that have gravity-dependent blood flow, due to edema, fibrosis, and hyperkeratosis, especially lower extremities. Among the various conditions that can induce chronic lymphedema are tumors, trauma, radiotherapy, obesity, hypothyroidism, chronic venous stasis, and AIDS-related Kaposis sarcoma. Kaposis sarcoma is a vascular tumor associated with the presence of human gammaherpesvirus 8 that is predominantly cutaneous, locally aggressive, with metastasis, and is associated with the production of factors that favor inflammation, lymphatic obstruction, and lymphedema.

Diagnosis of elephantiasis nostras verrucosa as a clinical mani-festation of Kaposi's sarcoma / Diagnóstico de elefantiasis nostras verrucosa como mani-festación clínica del Sarcoma de Kaposi

Elephantiasis nostras verrucosa, a rare manifestation of Kaposi's sarcoma, is a progressive cutaneous hypertrophy caused by chronic non-filarial lymphedema secondary to obstruction of the lymphatic system that can lead to severe disfigurement of parts of the body that have gravity-dependent blood flow, due to edema, fibrosis, and hyperkeratosis, especially lower extremities. Among the various conditions that can induce chronic lymphedema are tumors, trauma, radiotherapy, obesity, hypothyroidism, chronic venous stasis, and AIDS-related Kaposi's sarcoma. Kaposi's sarcoma is a vascular tumor associated with the presence of human gammaherpesvirus 8 that is predominantly cutaneous, locally aggressive, with metastasis, and is associated with the production of factors that favor inflammation, lymphatic obstruction, and lymphedema.

Acrodermatitis continua de Hallopeau y artritis psoriática: reporte de caso / Acrodermatitis continua of Hallopeau and psoriatic arthritis: a case report

Un hombre de 44 años, previamente sano, consultó por poliartralgias asimétricas de características inflamatorias de tres meses de evolución, que comprometió pequeñas y grandes articulaciones. Poco tiempo después desarrolló pústulas periungueales sobre una base eritematosa y degeneración progresiva de la lámina ungueal del pulgar izquierdo, limitando severamente su funcionalidad. Destacó al examen físico la presencia de placas eritemato-escamosas y pustulosas bien delimitadas en el primer y tercer dedo de la mano izquierda con onicodistrofia severa, que fue un aspecto clave para establecer el diagnóstico de acrodermatitis continua de Hallopeau. Adicionalmente, se observaron otros hallazgos del espectro psoriático: parches eritematosos en el escroto, placas eritemato-escamosas con costras hemorrágicas en ambas rodillas y dactilitis. Se confirmó histológicamente el diagnóstico de psoriasis inversa, psoriasis en placas y psoriasis pustular, respectivamente y con los criterios de Classification Criteria for Psoriatic Arthritis, CASPAR, el diagnóstico de artritis psoriática.

A 44-year-old man, previously healthy, consulted for a three-month history of asymmetrical polyarthralgia with inflammatory features involving small and large joints. A few days later, he developed erythema covered by pustules in the nail folds and progressive degeneration of the nail plate of the left thumb, with severe functional limitation. The physical exam showed well-defined erythematous scaly and pustular plaques in the first and third fingers of the left hand, with severe onychodystrophy, which was a key aspect in the diagnosis of acrodermatitis continua of Hallopeau. Other signs of the psoriatic spectrum were observed: erythematous patches of the scrotum, erythematous scaly plaques with hemorrhagic crusts on both knees, and dactylitis. The diagnosis of inverse psoriasis, plaque psoriasis, and pustular psoriasis, respectively, were confirmed by histopathology and, with the CASPAR criteria, psoriatic arthritis. This case is of particular dermatological interest due to the variety of psoriatic manifestations recognized in a single patient and because of the poorly described association between acrodermatitis continua of Hallopeau with psoriatic arthritis.

Malignant acanthosis nigricans as a paraneoplastic manifestation of metastatic breast cancer.

Malignant acanthosis nigricans is a rare paraneoplastic syndrome, usually associated with a gastric adenocarcinoma and less frequently with other neoplasms. In general, its appearance indicates a poor prognosis with a survival of < 2 years. We describe the case of a 40-year-old patient who presented with generalized cutaneous thickening that had a velvety appearance, was rapidly progressing, and involved right axillary adenopathy. Skin and nipple biopsy yielded results consistent with acanthosis nigricans, and palpable adenopathy biopsy results were compatible with mammary adenocarcinoma (human epidermal growth factor receptor 2 positive and estrogen and progesterone receptor negative) without a detectable primary tumor. This case of malignant acanthosis nigricans is presented because of the importance of its early recognition as a paraneoplastic syndrome and its relation with mammary adenocarcinoma, an association infrequently reported in the literature.

Úlcera de Lipschütz, un diagnóstico para considerar en la población pediátrica / Ulcer of Lipschütz, a diagnosis to consider in the pediatric population

La úlcera vulvar aguda o úlcera de Lipschütz corresponde a una entidad poco frecuente, por lo general, subdiagnosticada. Se caracteriza por la aparición súbita o aguda de lesiones ulcerosas y dolorosas en la vulva, la vagina y/o el periné, sin antecedente de contacto sexual en niñas y adolescentes. Su etiología permanece desconocida en la mayoría de los casos, aunque se ha asociado a agentes infecciosos. Para su estudio, se deben sospechar infecciones de transmisión sexual, reacciones adversas a medicamentos, enfermedades autoinmunes e inmunosupresión. Las lesiones ulcerosas desaparecen espontáneamente, de manera habitual, sin secuelas ni recurrencias a largo plazo. Se presenta el caso de una paciente adolescente de 11 años, sin inicio de actividad sexual ni menarquia y que consultó por un cuadro prodrómico de cuatro días, seguido del desarrollo agudo de úlceras genitales. Se discuten sus causas, presentación clínica, diagnósticos diferenciales y tratamiento.

Acute vulvar ulcer or ulcer of Lipschütz corresponds to a rare disease, being usually underdiagnosed. It is characterized by a sudden or acute development of ulcerous and painful lesions in the vulva, vagina and/or perineum, without a previous history of sexual contact in girls and teenagers. Its etiology remains unknown in most cases, although they have been associated with infectious agents. Their study includes the suspicion of sexually transmitted infections, adverse drug reactions, autoimmune diseases and immunosuppression. The ulcerative lesions disappear spontaneously, usually without sequelae or recurrences in the long term. We present the case of an 11-year-old adolescent patient, without sexual initiation, or menarche and who consulted due to a four-day prodromal condition, followed by the acute development of genital ulcers. Its causes, clinical presentation, differential diagnosis and treatment are discussed.

[A case report of granuloma faciale, an uncommon cutaneous vasculitis]. / Reporte de caso de granuloma facial, una vasculitis cutánea infrecuente.

Granuloma faciale is an uncommon benign dermatosis, with unknown etiology, usually asymptomatic, characterized by chronic inflammation localized in sun-exposed areas of the face with a characteristic histological pattern. Although response to treatment is variable, there are multiple therapeutic alternatives that have been reported to be effective in some patients, including systemic treatments with steroids and dapsone or also topical treatments like intralesional corticosteroid, cryotherapy and calcineurin inhibitors, such as tacrolimus. We present the case of an adult patient with an asymptomatic erythematous-violet plaque on the right cheek, with progressive slow growth over two years, clinically and histologically and pathologically compatible with a facial granuloma. The patient responded well to intralesional corticosteroids.

El granuloma facial es una dermatosis benigna poco frecuente de etiología desconocida, generalmente asintomática, caracterizada por inflamación crónica y localizada habitualmente en zonas fotoexpuestas de la cara, con un patrón histológico característico. Si bien la respuesta al tratamiento es variable, existen múltiples opciones terapéuticas que han reportado ser efectivas en algunos pacientes. Entre estas alternativas se incluyen tratamientos sistémicos con corticoides y dapsona o tratamientos intralesionales con corticoides, crioterapia e inhibidores de calcineurina tópicos. Describimos el caso de un paciente adulto con una placa eritemato violácea asintomática en mejilla derecha, de crecimiento lentamente progresivo de dos años de evolución, clínica e histopatológicamente compatible con granuloma facial y respuesta favorable a corticoides intralesionales.

Síndrome de DRESS con compromiso pulmonar extenso: una presentación inhabitual / Drug rash with eosinophilia and systemic symptoms or DRESS syndrome: report of one case

Drug rash with eosinophilia and systemic symptoms or DRESS Syndrome is a rare, serious and potentially fatal adverse drug reaction. It is characterized by widespread morbilliform and edematous skin lesions, associated with eosinophilia, lymphadenopathy and internal organ involvement and unusually associated with pulmonary symptoms. We report a 47-year-old male with DRESS syndrome, manifested with typical skin lesions and extensive pulmonary involvement, responding satisfactorily to systemic corticosteroids.

Enfermedades acantolíticas: caracterización de pacientes con diagnóstico de enfermedad de Hailey-Hailey, enfermedad de Darier y enfermedad de Grover, entre los años 2007 y 2017 en el Hospital Clínico San Borja Arriaran y revisión de la literatura / Characterization of patients diagnosed with Hailey-Hailey disease: Darier's disease and Grover's disease, between 2007 and 2017 at the San Borja Arriaran Clinical Hospital and review of the literature

INTRODUCCIÓN: Las enfermedades acatólicas son un grupo heterogéneo de enfermedades que presentan como característica central histopatológica la acantosis. Generalmente presentan un curso de evolución crónica y recidivante, con variadas manifestaciones clínicas. OBJETIVO: caracterizar los pacientes con diagnóstico de enfermedad acantolítica, bajo 5 criterios clínicos y realizar una revisión de la literatura. MÉTODOS: Se realizó una revisión de la base de datos del Servicio Anatomía Patológica del Hospital San Borja Arriaran (HSBA) entre los años 2007 y 2017 y se complementaron con los antecedentes clínicos extraídos de las fichas clínicas.RESULTADOS: Se obtuvo un total de 13 casos. el 53,8% correspondieron a enfermedad de Darier, 20,6% a enfermedad de Hailey-Hailey y un 20,6% a enfermedad de Grover, obteniendo un promedio de edad al momento del diagnóstico de 22,5 años, 44,3 años y 47,6 años respectivamente. Los antecedentes familiares estuvieron presentes en el 53,8% del total de pacientes, ninguno de ellos presentaba estudio genético. El 61,5% de la muestra correspondió a pacientes de sexo femenino y el promedio de años de evolución previo al diagnóstico fue de 7,4 años para Darier, 8,6 para Hailey-Hailey y para Grover. El 100% de los pacientes con enfermedad de Darier y Grover estaban con terapia sistémica y el 66,6% de enfermedad de Hailey-Hailey con terapia tópica, todos con adecuada respuesta clínica. DISCUSIÓN y conclusiones: las enfermedades acantolíticas corresponden a genodermatosis poco frecuente cuyo diagnóstico y tratamiento constitu-yen un desafío para el dermatólogo

INTRODUCTION: Atytolic diseases are a heterogeneous group of diseases that present acanthosis as a histopathological central characteristic. They usually present a course of chronic and recurrent evolution, with varied clinical manifestations. OBJECTIVE: to characterize the patients diagnosed with acantholytic disease, with 5 clinical criteria and to carry out a review of the literature. METHODS: A review of the database of the Pathological Anatomy Service of the San Bor-ja Arriaran Hospital (HSBA) between 2007 and 2017 was carried out and complemented with the clinical records extracted from the clinical files. RESULTS: A total of 13 cases were obtained. 53.8% corresponded to Darier's disease, 20.6% to Hailey-Hailey's disease and 20.6% to Grover's disease, obtaining an average age at diagnosis of 22.5 years, 44.3 years and 47.6 years respectively. Family history was present in 53.8% of the to-tal patients, none of them had a genetic study. 61.5% of the sample corresponded to female patients and the average of years of evolution prior to diagnosis was 7.4 years for Darier, 8.6 for Hailey-Hailey and for Grover. 100% of the pa-tients with Darier and Grover's disease were on systemic therapy and 66.6% of Hailey-Hailey's disease with topical therapy, all with adequate clinical response. DISCUSSION AND CONCLUSIONS: acantholytic diseases correspond to rare genodermatosis whose diagnosis and treatment constitute a challenge for the dermatologist.