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.

Clinical analysis of the radiotherapy for Langerhans cell histiocytosis in the head and neck region / 中国癌症杂志

Background and purpose: Langerhans cell histiocytosis (LCH) is a rare complex reticulocndotheliai disease that often invades the head and neck. There in no consensus of treatment. Radiation is one of the treatment options for the localized lesions. Our aim in the study was to analyze the radiotherapeutic efficacy for LCH in the head and neck region. Methods: 8 patients with eosinophilic granuloma (EG) and 1 case with Hand-Schuller-Christian disease (HSC) were treated with radiotherapy in our hospital from July 2000 to May 2007. Their clinical data were retrospectively analyzed. Results: 5 cases of EG were treated with tumor partial excision and radiation, while the other 3 cases of EG were given radiation alone after biopsy. The HSC was administered with radiation and endocrine therapy. All the patients were followed up for 1.5-8 years. The response rate was 89%. Conclusion: Radiotherapy of LCH is an effective modality, but the optimal dose needs to be further studied.

Langerhans Cell Histiocytosis in the Juvenile Mandible

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.