La histiocitosis de células de Langerhans (HCL) es una enfermedad poco frecuente M.A. TossSurvey of Hand-Schuller- Christian’disease in otolaryngology. Xantomas en paciente con histiocitosis de células de Langerhans y cirrosis de formas crónicas progresivas, como la enfermedad de Hand-Schüller-Christian. Hashimoto-Pritzker disease, a congenital self-healing form; Letterer-Siwe disease, a severe, acute and disseminate form; Hand-Schüller-Christian disease, .

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LHH, Letterer-Siwe hastaligi, Hand-Schuller-Christian hastaligi, eozinofilik granulom ve konjenital kendiliginden iyilesen retikulohistiyositoz adli dort belirgin formdan olussa da bazen ortusme sendromlari seklinde de ortaya cikabilmektedir. CT scan showed a 3.

By using this site, you agree to the Terms of Use and Privacy Policy. CT may be useful to define the extent of the process.

Langerhans cell histiocytosis in the maxillofacial area in adults: Report of three cases

Views Read Edit View history. A rationale for diagnosis and treatment. Langerhans cells are oval or rounded in shape, pale, and have a predominantly eosinophilic cytoplasm 2,8. Langerhans cell histiocytosis is most common in children and young adults. Letterer-Siwe definition of Letterer-Siwe by Medical dictionary https: The lesions may be well defined or poorly defined with or without associated sclerotic borders.

Nine years post-surgery, there are no signs of recurrence of the lesion. As a result of their common underlying histopathology, Lichtenstein grouped these diseases together under the name of histiocytosis X.

One and a half years following surgery, there are no signs of relapse, nor complications associated with LCH.

Hand–Schüller–Christian disease

Clinical History The patient presented with local pain, swellingtenderness of the thigh, and loose teeth. Langerhans cell histiocytosis restricted to the oral mucosa. Radiol Clin North Am.


Prognosis of LCH in adults is generally good due to the slow evolution of the disease and its favourable response to treatment Related Topics in Histiocytosis. The lesion was elastic to the touch, painful, and impeded normal upper eyelid movement Fig. For example, the rate of recurrence of the disease falls markedly with 6-month treatments of prednisone and vinblastine. Letterer-Siwe disease acute disseminated diseaseHand-Schuller Christian disease multifocal or uni-focal diseaseand Eosinophilic granuloma usually uni-focal disease Table.

The etiopathogenesis of LCH is unknown, although it appears to be linked to a disturbance in immune system regulation 2,3,8, The clinical course is schu,ler related to the number of organs affected at presentation. In the past, LCH was subdivided into three categories: This cutaneous condition article is a stub.

Some clinicians advocate aggressive, generalized treatment in these cases, whereas others suggest a conservative, symptomatic approach, unless there are systemic manifestations such as pain, fever, failure to thrive or disorders of vital organs.

A year-old male was referred to the Dept. The differential diagnosis includes: CT of the femur soft tissue window. Biopsy revealed the diagnosis of LCH.

Lateral view of the skull: Granuloma eosinofilo craneal solitario. Figure 1 Plain radiographs Figure 1a. Langerhans cell histiocytosis in the maxillofacial area in adults. J CranioMaxillofac Surg ; Infobox medical condition new All stub articles. In the majority of refractory or life-threatening cases, there is lung involvement 11with a worse prognosis for isolated lung affectation than for multisystemic disease, even if there are lung lesions in the latter case 4.

In the skull, lesions usually have sharply defined borders with uneven involvement of the inner and outer table. It is associated with a triad of exophthalmoslytic bone lesions often in the skulland diabetes insipidus from pituitary stalk infiltration.


CT of the pelvis osseous window: Por esta razon, a la forma multisistemica diseminada y aguda de HCL se le denomino enfermedad de Hand-Schuller-Christian, y a la forma multifocal, progresiva y cronica, enfermedad de Letterer-Siwe. N Engl J Med ; Diffuse Histiocytosis X involving the eyelid of year-old Woman.

Diagnosis is made by means of histopathological analysis, and imaging studies are necessary in order to determine extent of the disease. Related Bing Images Extra: Finally, the disease may involve the liver, spleen, lymph nodes, skin, and lungs.

Langerhans cell aggregates are nodular, especially in the lungs, and are variably accompanied by eosinophils, foamy cells, neutrophils, and fibrosis. InLichtenstein observed cytoplasmic bodies, known as X bodies, within histiocytes from tissues of patients suffering from what were previously considered distinct clinical disorders: Pain and swelling of the mandible, with mobility and loss of teeth, may be the presenting symptoms of the disease 1,3,6,8,11, Trans Am Ophthalmol Soc ; Eosinophilic granuloma of the orbit: Granulomatous and lymphoproliferative diseases of the Head and Neck.

After en bloc resection of the tumor, the orbital walls were reconstructed with outer-table calvarial bone grafts. Final Diagnosis Hand-Schuller-Christian disease. A neoplastic proliferation of Langerhans cells which contain Birbeck granules by ultrastructural examination. The soft tissue mass accompanying all osseous lesions can best be seen with CT scan and MRI 3the latter showing a well-defined area of soft tissue surrounding a focal lesion, with altered bone marrow signal 2.