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, .

Author: Araran Kagacage
Country: Martinique
Language: English (Spanish)
Genre: Business
Published (Last): 20 May 2008
Pages: 132
PDF File Size: 6.16 Mb
ePub File Size: 11.10 Mb
ISBN: 870-6-37012-440-2
Downloads: 84258
Price: Free* [*Free Regsitration Required]
Uploader: Gulrajas

Maxillofacial manifestations of Langerhans cell histiocytosis: The clinical course is generally related to the number of organs affected at presentation.

There are no controlled trials proposing an optimal treatment protocol for LCH. Clinical History The patient presented with local pain, swellingtenderness of the thigh, and loose teeth.

In the skull, lesions usually have sharply defined borders with uneven involvement of the inner and outer table. Retrieved from ” https: On the orthopantomogram, new cystic lesions were observed in the regions of dental pieces 25, 36 to 37, and 46 to 48, with affectation of these teeth Fig. Department of Oral and Maxillofacial Surgery. When yet more new lesions were discovered in the right mandibular angle, ramus and body Fig. As a result of their common underlying histopathology, Lichtenstein grouped these diseases together under the name of histiocytosis X.

Letterer-Siwe | definition of Letterer-Siwe by Medical dictionary

From Wikipedia, the free encyclopedia. A group of rare disorders in which too many Langerhans cells a type of white blood cell grow in certain tissues and organs including the bones, skin, and lungs, and damage them.


Albacete 2 Head of Section.

Bone scintigraphy showed no other lesions. The lesion was elastic to the touch, painful, and impeded normal upper eyelid movement Fig. Langerhans cells are oval or rounded in shape, pale, and have a predominantly eosinophilic cytoplasm 2,8. For example, the rate of recurrence of the disease falls markedly with 6-month treatments of prednisone and vinblastine. Intraorbital involvement can produce proptosis 3,8as occurred in Case 2 of this report. CT of the pelvis soft tissue window: Oral Langerhans cell histiocytosis.

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, Radiographic imaging showed a lobulated, lytic lesion in the right fronto-supraorbital region, with ground-glass appearance.

Plain radiographs show LCH bony lesions as non-calcified, lytic areas without peripheral sclerosis. The postoperative histopathological report revealed LCH. Resection was performed by superolateral orbitotomy, using a combined craniofacial approach.

enfermedad de Hand-Schüller-Christian

Hematology and Oncology Chapters. La Histiocitosis X incluye tres componentes: A multifocal, unisystem form of Langerhans-cell histiocytosis. Computer tomography of the pelvis and femur showed chriatian leasions with disruption of the cortex and small soft tissue mass.

Imaging Findings The patient presented with local pain, swellingtenderness of the thigh, and loose teeth. The lesions may also appear as permeative lesions with ill-defined borders and periosteal reaction. Search other sites for ‘Hand-Schuller-Christian Syndrome’.

There was a problem providing the content you requested

Churchill Livingstone, London Although lesions may appear in tissues of varying origins such as skin, hypothalamus, liver, lung, or lymphoid tissue 4,6,8,11,12bone is the most common site of the disease 3, Oral mucosa affectation, although infrequent, is characterized by gingival hypertrophy and ulcers of the buccal mucosa, hard and soft palates, and tongue 8,10, Quintessence Publishing Co, Inc; Discussion Chrostian disease is a chronic disseminated form of histiocytosis X, in which idiopathic non-neoplastic proliferation of histiocytes occurs.


Related Topics jand Histiocytosis. Langerhans cell histiocytosis in the cshuller area in adults. The differential diagnosis of mandibular lesions must include odontogenic cysts and tumors, primary bone tumors, osteomyelitis, metastases, multiple myeloma and giant cell granuloma 2,11, A rationale for diagnosis and treatment.

On MRI examination, T1-weighted images reveal a lesion isointense to adjacent tissue and T2-weighted images reveal high signal areas of marrow replacement. This cutaneous condition article is a stub. Langerhans cell histiocytosis, LCH, histiocytosis X, lymphoproliferative disease. Langerhans cell histiocytosis is most common in children and young adults.

The overall 5-year survival rate for LCH is approximately Related links to external sites from Bing. Initial stage lesions tend to be very cellular, whereas in more advanced stages, a higher degree of fibrosis is present enfrrmedad.