[Chronic cough, pleuritic chest pain, and night sweats in a 45­year-old female smoker]. / Chronischer Husten, atemabhängiger Thoraxschmerz und Nachtschweiß bei einer 45­jährigen Raucherin.

A 45-year-old woman presented with chronic cough, pleuritic chest pain, and night sweat. High-resolution computed tomography revealed multiple bilateral nodular lesions in a centrilobular distribution, primarily located in the upper and mid lung zones with relative sparing of the lung bases. No lymphadenopathy or pleural effusions were detected. Histological analysis confirmed the suspected diagnosis of pulmonary Langerhans cell histiocytosis. After smoking cessation the patient recovered completely.

Histiocitose de células de Langerhans diagnosticada em um paciente de idade avançada / Langerhans cell histiocytosis diagnosed in an elderly patient

A histiocitose de células de Langerhans é uma doença rara caracterizada proliferação de células de Langerhans. Neste artigo descrevemos um caso de histiocitose de células de Langerhans em um paciente de 63 anos, com uma lesão expansiva periorbital como primeiro sintoma e cuja tomografia computadorizada revelou acometimento pulmonar característico da doença. A condução do caso, os achados radiológicos e os resultados são apresentados.

Langerhans cell histiocytosis is a rare disease characterized by proliferation of Langerhans cells. We report a case of Langerhans cell histiocytosis in a 63-year-old patient, who presented an expansile periorbital lesion as the first symptom, in whom computed tomography revealed characteristic lung commitment of the disease. The case management, as well as radiological findings and outcomes are described.

Histiocitosis de células de Langerhans en menores de un año / Langerhans cell histiocytosis in children under one year

Introducción. La histiocitosis de células de Langerhans se debe a la proliferación clonal de histiocitos activados que invaden diversos tejidos. Se produce en todas las edades, desde el nacimiento hasta la adultez, con un pico de mayor incidencia entre 1 a 4 años. Objetivo. Describir las características de 15 pacientes pediátricos menores de 1 año con diagnóstico de histiocitosis de células de Langerhans, las manifestacionesclínicas y evolución de la enfermedad. Métodos. Estudio descriptivo retrospectivo, realizado en el Hospital Ramos Mejía y el Hospital Alemán, entre 1999 y 2007. Resultados. Se revisaron las historias de 15 pacientes, 6 niñas y 9 niños. Las lesiones en 8 casos estaban presentes al nacer y 7 aparecieron entre los 2 y 12 meses de edad. En las congénitas se observó como única manifestación clínica inicial el compromiso cutáneo. Uno de estos pacientes presentó evolución sistémica, con compromiso pulmonar, hepático y esplénico, actualmente en tratamiento. De los pacientes con lesiones posteriores al nacimiento, sólo 3 tenían compromiso cutáneo únicamente, el resto presentó compromiso sistémico. Uno de estos pacientes falleció durante el tratamiento. En la histopatología se observaron histiocitos en la dermis papilar con marcado epidermotropismo. La inmunomarcación con CD1a y S100 fue positiva. Conclusiones. La histiocitosis de células de Langerhans es una entidad con dos variantes: la forma congénita y la de inicio posterior al nacimiento, ambas con capacidad para producir compromiso sistémico.

Introduction. Langerhans cell histiocytosis is characterized by a clonal proliferation of activated Langerhans cells that infiltrate various organs of the body. Occurs at any age, from newborn until adulthood, with an incidence peak at 1-4 years. Objective. To describe the morphologyc characteristics of skin lesions and clinical course of 15 patients with Langerhans cell histiocytosis. Methods. A retrospective review of the medical records of patients with Langerhans cell histiocytosis from Ramos Mejia Hospital and Aleman Hospital, between 1999-2007. Results. Review of medical records from 15 patients, 6 females and 9 males. Skin lesions were congenital in 8 cases and appeared between 2-12 months of age in 7 cases. The patients with congenital presentation only had a cutaneous manifestation; one patient who developed a systemic compromise (lung, liver and spleen) is currently under treatment. Three patients with presentation after birth only had cutaneous lesions, the others had a sistemic disease. One of this patients died during treatment. Histopathology showed a histiocytic infiltrate in the papilary dermis with epidermotrophism; inmunomarking with S100 and CD1a was positive. Conclusion. Both clinical manifestation (congenital and after birth) represent different ends of a spectrum of the same condition, with the potencial to develop into disseminated Langerhans cell histiocytosis.

Histiocitosis de células de Langerhans en menores de un año / Langerhans cell histiocytosis in children under one year

Introducción. La histiocitosis de células de Langerhans se debe a la proliferación clonal de histiocitos activados que invaden diversos tejidos. Se produce en todas las edades, desde el nacimiento hasta la adultez, con un pico de mayor incidencia entre 1 a 4 años. Objetivo. Describir las características de 15 pacientes pediátricos menores de 1 año con diagnóstico de histiocitosis de células de Langerhans, las manifestacionesclínicas y evolución de la enfermedad. Métodos. Estudio descriptivo retrospectivo, realizado en el Hospital Ramos Mejía y el Hospital Alemán, entre 1999 y 2007. Resultados. Se revisaron las historias de 15 pacientes, 6 niñas y 9 niños. Las lesiones en 8 casos estaban presentes al nacer y 7 aparecieron entre los 2 y 12 meses de edad. En las congénitas se observó como única manifestación clínica inicial el compromiso cutáneo. Uno de estos pacientes presentó evolución sistémica, con compromiso pulmonar, hepático y esplénico, actualmente en tratamiento. De los pacientes con lesiones posteriores al nacimiento, sólo 3 tenían compromiso cutáneo únicamente, el resto presentó compromiso sistémico. Uno de estos pacientes falleció durante el tratamiento. En la histopatología se observaron histiocitos en la dermis papilar con marcado epidermotropismo. La inmunomarcación con CD1a y S100 fue positiva. Conclusiones. La histiocitosis de células de Langerhans es una entidad con dos variantes: la forma congénita y la de inicio posterior al nacimiento, ambas con capacidad para producir compromiso sistémico.(AU)

Introduction. Langerhans cell histiocytosis is characterized by a clonal proliferation of activated Langerhans cells that infiltrate various organs of the body. Occurs at any age, from newborn until adulthood, with an incidence peak at 1-4 years. Objective. To describe the morphologyc characteristics of skin lesions and clinical course of 15 patients with Langerhans cell histiocytosis. Methods. A retrospective review of the medical records of patients with Langerhans cell histiocytosis from Ramos Mejia Hospital and Aleman Hospital, between 1999-2007. Results. Review of medical records from 15 patients, 6 females and 9 males. Skin lesions were congenital in 8 cases and appeared between 2-12 months of age in 7 cases. The patients with congenital presentation only had a cutaneous manifestation; one patient who developed a systemic compromise (lung, liver and spleen) is currently under treatment. Three patients with presentation after birth only had cutaneous lesions, the others had a sistemic disease. One of this patients died during treatment. Histopathology showed a histiocytic infiltrate in the papilary dermis with epidermotrophism; inmunomarking with S100 and CD1a was positive. Conclusion. Both clinical manifestation (congenital and after birth) represent different ends of a spectrum of the same condition, with the potencial to develop into disseminated Langerhans cell histiocytosis.(AU)

Drug therapy for the treatment of Langerhans cell histiocytosis.

Langerhans cell histiocytosis results from the abnormal accumulation of a class of dendritic cells normally found in the skin, which proliferate in many organ systems along with lymphocytes, macrophages and eosinophils. Standard therapy for Langerhans cell histiocytosis includes vinblastine and prednisone with or without methotrexate and mercaptopurine, depending on the extent of disease. Effective therapies for patients unresponsive to the above include cytosine arabinoside and cladribine. Thalidomide has proven useful for patients with Langerhans cell histiocytosis of the skin and/or bone. Emerging therapies include the use of monoclonal antibodies against the CD1a or CD52 epitopes found on Langerhans cells. Specific therapies directed against the cytokines that are apparently critical to the abnormal proliferation have not yet been defined.