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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Search other sites for ‘Hand-Schuller-Christian Syndrome’. A year-old male was referred to the Dept. When lymphatic nodes are involved, the most adequate treatment is excision of affected nodes. In the spine, typically, there is lysis of affected vertebrae leading to progressive collapse vertebra plana with preservation of adjacent disc space, which usually allows distinction from infection.

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Adult Langerhans Cell Histiocytosis. The postoperative histopathological report revealed LCH. TavernarakiFeb InLichtenstein observed cytoplasmic bodies, known as X bodies, within histiocytes from tissues of patients suffering from what were previously enfermeadd distinct clinical disorders: Granulomatous and lymphoproliferative diseases of the Head and Neck. Back Links pages that link to this page.

enfermedad de Hand-Schüller-Christian

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, Cystectomy of mandibular lesions was performed, as enfermdead as exodontia of dental pieces 36, 37, 46, 47 and 48 due to mobility. An orthopantomogram showed extensive bone loss with respect to previous radiographs, involving right maxillary mesial surface of 17 to distal surface of 12 Fig.


Quintessence Publishing Co, Inc; The lesions may also appear as permeative lesions with ill-defined borders and periosteal reaction. Histological appearance does not correlate with clinical behaviour La Histiocitosis X incluye tres componentes: Biopsy revealed the diagnosis of LCH.

Fundamentals of skeletal radiology. Revision de la literatura.

La histiocitosis de celulas de Langerhans, tambien conocida como sindrome X, granuloma eosinofitico, enfermedad de Letterer-Siwe y enfermedad de Hand-Schutter-Christian es una enfermedad protiferativa que se caracteriza por la acumulacion irregular y localizada de cetutas en cualquier tugar det organismo 1.

Given the rarity of LCH, hxnd strongly recommend protocolization of treatment.

enfermedad de Hand-Schüller-Christian

Arch Otolaryngol Head Neck Surg ; Bone scintigraphy showed a single, hypercaptating lesion in the right supraorbital region. Bone scintigraphy showed no other lesions. A rationale for diagnosis and treatment. Oral Langerhans cell histiocytosis. There was no evidence of periosteal reaction or marginal sclerosis. In the maxillofacial area, skin affectation may appear as a papular rash; scalp involvement has a seborrhoea-like presentation. Langerhans cell histiocytosis restricted to the oral mucosa.

Eosinophilic granuloma of the orbit: Four months following the second surgery, the patient presented with trismus, swelling and pain in the left upper jaw and in right and left mandibular bodies.


To further this end, patients ce from this disease, particularly those with multiorganic affectation, should be included in clinical trials carried out by the Histiocytosis Society. Letterer-Siwe definition of Letterer-Siwe by Medical dictionary https: Discussion The etiopathogenesis of LCH is unknown, although it appears to be linked to a disturbance in immune system regulation 2,3,8, Case Hand-Schuller-Christian disease Author s.

Plain radiographs of the skull showed large lesions, giving the chrisian of “geographic skull” and “floating teeth”. There is involvement of multiple sites in one organ system, most frequently the bone.

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, Juvenile xanthogranuloma Hemophagocytic lymphohistiocytosis Erdheim-Chester disease Niemann—Pick disease Sea-blue histiocyte Benign cephalic histiocytosis Generalized eruptive histiocytoma Xanthoma disseminatum Progressive nodular histiocytosis Papular xanthoma Hereditary progressive mucinous histiocytosis Reticulohistiocytosis Multicentric reticulohistiocytosisReticulohistiocytoma Indeterminate cell histiocytosis.

In the past, LCH was subdivided into three categories: