Histiocitosis dendrocítica (histiocitosis de células de langerhans), manifestaciones orales y craneofaciales: reporte de tres casos / Dendrocitic histiocytosis (langerhans cell histiocytosis), oral and craniofacial manifestations: report of three cases

La Histiocitosis de células de Langerhans (HCL) corresponde a una proliferación anormal de células dendríticas, de tipo clonal, cuyo espectro clínico general incluye compromiso de la piel y las mucosas, las uñas, el hueso, la médula ósea, el hígado, el bazo, linfonodos, el pulmón, el tracto gastrointestinal inferior, el sistema endocrino y el sistema nervioso central. En este trabajo presentamos tres casos de la enfermedad, con manifestaciones orales y craneofaciales, analizadas desde el punto de vista clínico (examen extra e intra oral), imagenológico (tomografías computadas) e histopatológico (expresión de marcador específico CD1a). Dos casos fueron clasificados como HCL de presentación aguda diseminada y uno como presentación crónica. Los pacientes fueron tratados oportunamente con quimioterapia según el protocolo del Programa Infantil Nacional de Drogas Antineoplásicas.

The Langerhans cell histiocytosis (LCH) corresponds to an abnormal proliferation of dendritic cells, clonal type, which usually involves compromise of skin and mucous membranes, nails, bone, bone marrow, liver, spleen, lymph nodes, lung, lower gastrointestinal tract, endocrine system and the central nervous system. We present three cases of the disease, with oral and craniofacial manifestations, analyzed from the clinical perspective (intra and extra oral exam), imaging (CT scans) and histopathological (specific marker CD1a expression). Two cases were classified as acute disseminated LCH presentation and one as a chronic disease. Patients were treated with chemotherapy timely according to the protocol of the National Child Program of Antineoplastic Drugs.

Histiocitosis de células de langerhans-síndrome de letterer-siwe: afectación de piel, tejido linfoide, hígado, bazo y hueso: presentación de caso / Langerhans cells histiocytosis, letterer-siwe syndrome: skin, lymphoid tissue, liver, spleen and bone commitment: case report

En este artículo se presenta el caso de una paciente en edad pediátrica, a quien se le diagnostic el síndrome de Letterer-Siwe por hallazgos clínicos, de imagen e histopatológicos, que evidenciaron una afectación multiorgánica por infiltración histiocitaria. Este síndrome es una entidad inusual y agresiva, parte del espectro clínico de las histiocitosis de células de Langerhans. Tiene el peor pronóstico de los tres tipos conocidos (granuloma eosinófilo, Hand-Schüller-Christian y Letterer- Siwe), por un compromiso infiltrativo más extenso, porque afecta un mayor número de órganos y porque tiende a aparecer en edades más tempranas. Los hallazgos clínicos de los síndromes que constituyen la histiocitosis de células de Langerhans están directamente relacionados con las alteraciones funcionales de los órganos afectados. En los casos crónicos, los pacientes evolucionan de forma rápida y progresiva a falla multiorgánica y con frecuencia fallecen por cuadros sépticos. Las imágenes diagnósticas son útiles; sin embargo, la apariencia de los hallazgos habitualmente no es específica, por lo que es necesario el estudio histológico.

A case of a female patient at pediatric age, who has been diagnosed with Letterer-Siwe is presented in this article. The diagnosis was based on clinical, imaging and histopatologic findings that exhibit multiorgan commitment caused by histiocytic infiltration. This syndrome is a rare andaggressive entity which is part of the Langerhans cell histicytosis clinical spectrum. It presents the worst prognosis of the three known types (eosinophilic granuloma, Hand-Schüller-Christian, and Letterer-Siwe syndromes) since it exhibits more extensive infiltrative comprise, involves a large number of organs and tends to occur at younger ages. The clinical findings of the syndromes thatconstitute Langerhans cells histiocytosis are directly related to functional alterations of the affected organs. In severe cases, patients promptly and progressively evolve into an organ failure and often die for septic issues. Diagnostic imaging is useful to see the compromise of most organs, howeverthe appearance of findings is usually not specific, so it is necessary a histological study.

Langerhans Cell Histiocytosis in the Juvenile Mandible

A Case of Letterer-Siwe Disease in Adult / 대한피부과학회지

Letterer-Siwe disease is a one of Langerhans cell histiocytosis and characterized by proliferation of Langerhans cells. It's clinical features are onset of infancy, hemorrhagic crusted papules and petechiae, hepatomegaly, lymphadenopathy, localized bone defect, and fatal outcome. We report a 38-year-old woman with hepatomegaly, lymphadenopathy, scaly papules and petechiae on the trunk. The eletronmicroscopy showed a tennis racquet shaped Birbeck granules in cytoplasm of histiocyte. Systemic treatment with etoposide, cyclophosphamide, prednisone, and vincristine was effective.

Three Cases of Letterer - Siwe Disease / 대한피부과학회지

Letterer-Siwe disease is one of Langerhans cell histiocytosis, with Hand-Schuller-Christian disease and eosinophilic granuloma, characterized by proliferation of Langevha is cell. The clinical course of Letterer-Siwe disease is acute fulmunant of casionally fatal, involving skin and many other internal organs, such as the lymph node, liver, spleen, ung and bone. We present 3 cases of Letterer-Siwe disease with characteristic cutaneous findings and revealed Langerhans granules by imrriunohistochemical stain and electronmicrc scopic examination. All patients died in spite of combined chernotherapy.

Histiocytosis-X with Giant Occipital Scalp Mass: A Case Report

Histiocytosis is a relatively rare disorder of the reticuloendothelial system involving the proliferation of histicoytes, granulation tissue, and inflammatory cells in many different organ systems1). Thus, the three manifestations of the same basic pathologic process:Eosinophilic granuloma, Hand-Schuller-Christian disease, and Letterer-Siwe disease have been classified as localized, chronic disseminated and acute disseminated histiocytosis-X. They were therefore included under the term histiocytosis-X and this concept has been generally accepted. The authors have experienced one case of histiocytosis-X, a rare disease. A 11 month-old femal patient presented with gradually enlarged palpable mass on the occipital area. The occipital skull was defected in a punched out fashion. The mass was completely removed. The pathologic findings revealed Histiocytosis-X and the patient was given chemotherapy.

Histiocytosis X: A Clinical Study / 대한정형외과학회잡지

Letterer-Siwe disease, Hand-Schuller-Christian disease and eosinophilic granuloma of the bone are merely different clinical expressions of one disease. Lichtenstein grouped these three clinical syndromes and proposed that this disease be termed histiocytosis X. This grouping was based on the histologic similarities and the possibility of transformation of one syndrome into another. The cause of histiocytosis X remains unknown. Histiocytosis X is a condition that presents single or multiple lesions, distributed in the soft tissue, lymph nodes, various organ and bones, especially in the areas of marked reticuloendothelial activity. The authors reviewed the clinical findings, radiographs and the treatment of the twenty-six patients with histiocytosis X diagnosed on the base of pathologic findings at Severance Hospital, between January, 1971 and December, 1980. Among the twenty-six patients, twenty-one patients could be followed, ranging from one month to six years, with an average follow-up of 1.6 years. The results obtained were as follows: 1. There was a slight male predominance (61.6%). The age ranged from 2 months to 42 years (average 8.2 years). Sixty-five percents of patients were the child under 4 years of age. 2. Hand-Schuller-Christian disease (50%) was the most common form of this disease. Letterer-Siwe disease developed in the youngest (average 1.2 years) and eosinophilic granuloma in the eldest (average 20.4 years). 3. The common manifestations were hepatomegaly, skin rashes and anemia in Letterer-Siwe disease; palpable mass, pain and exophthalmos in Hand-Schuller-Christian disease; pain and mass in eosinophilic granuloma. 4. The common sites of the skeletal lesions were skull, spine and femur, etc. 5. The patients with single skeletal lesion improved regardless of the methods of treatment. 6. Vinblastine, methotrexate and/or prednisone were valuable agents for the multiply involved patients. 7. Prognosis was poor for the patients who were young at onest, or who had multiple systemic involvements, but in case only with skeletal involvement the prognosis was good. Eosinophilic granuloma is the most benign variant, while Letterer-Siwe disease is the most malignant type.