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.