PDF | On Apr 1, , SANTIAGO MUZZO PONS and others published Enfermedad de hand Schuller Christian. Purpose/Material and Method: To describe the clinical case of a month-old boy with Hand-Schuller-Christian disease. Exophthalmos was the only systemic. Diabetes insipidus and growth retardation are common manifestations of hypothalamic involvement in patients with Hand-Schuller-Christian disease ( HSCD).

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Langerhans cell histiocytosis, LCH, histiocytosis X, lymphoproliferative disease. Views Read Edit View history.

Histological appearance does not correlate with clinical behaviour Finally, the chrixtian may involve the liver, spleen, lymph nodes, skin, and lungs. Radiographs of the femur and pelvis showed multiple lytic lesions with ill-defined borders, including one lesion of the acetabulum.

This page was last edited on 6 Julyat TI-weighted image with contrast: Defects in membranous bones, exophthalmos, and diabetes insipidus; an unusual syndrome of dyspituitarism. The classic triad is exophthalmos, diabetes insipidus and skull lesions.

Hand-Schuller-Christian Syndrome

Cystectomy of mandibular lesions was performed, as well as exodontia of dental pieces 36, 37, 46, 47 and 48 due to mobility.

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.

Eckardt A, Schultze A. A neoplastic proliferation of Langerhans cells which contain Birbeck granules by ultrastructural examination. Int J Dermatol ; After adequate workup to determine the extent of the disease, no other lesions were encountered. Symptoms Signs Labs Diagnosis Extra: The clinical course is generally related to the number of organs affected at presentation.


CT of the pelvis osseous window: Resolution of cutaneous lesions of histiocytosis Schuler by intralesion injections of interferon-beta. Radiographic imaging of lytic lesions of the skull reveals a punched-out pattern without evidence of periosteal reaction or marginal sclerosis, known as “geographic skull” 2, Started inthis collection now contains interlinked topic pages divided into a tree of 31 specialty books and chapters.

Current insights in a molecular age with emphasis on clinical oral and maxillofacial pathology practice. Comprehensive management of Head and Neck Tumors. Langerhans cell histiocytosis LCH is a disease of unknown etiology, characterized by proliferation of pathological Langerhans cells within different organs.

Plain radiographs show LCH bony lesions as non-calcified, lytic areas without peripheral sclerosis. Langerhans cell histiocytosis in the maxillofacial area christiann adults.

Enfermedad hand schuller christian pdf

There are no controlled studies establishing optimal treatment for LCH 2,7,9, On MRI examination, T1-weighted images reveal a lesion isointense to adjacent tissue and T2-weighted images reveal high signal areas of marrow replacement. Report of three cases. Langerhans-cell granulomatosis can involve a single organ, or can be a systemic disorder. Handschullerchristian disease is associated with multifocal langerhans cell histiocytosis. Definition CSP group of disorders of histiocyte proliferation which includes Letterer-Siwe disease; Hand-Schueller-Christian syndrome; uand eosinophilic granuloma; Langerhans cells are components of the lesions.

Final Diagnosis Hand-Schuller-Christian disease. Resection was performed by superolateral orbitotomy, using a combined craniofacial approach.

Hand-Schüller-Christian disease | Radiology Reference Article |

J CranioMaxillofac Surg ; After en bloc resection of the tumor, the orbital walls were reconstructed with outer-table calvarial bone xhristian. Neoplasm of the immune system.


It is associated with a triad of exophthalmoslytic bone lesions often in the skullebfermedad diabetes insipidus from pituitary stalk infiltration. In the united states, a rare disease is defined as one that affects fewer thanpeople.

Monocyte- and macrophage-related cutaneous conditions Medical triads Cutaneous condition stubs.

Subsequently, two additional surgeries were undertaken due to the appearance of new lesions in the right malar bone, right and left christisn, and left mandibular body Fig. 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.

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The overall 5-year survival rate for LCH is approximately Diagnosis Skin biopsy Bone Marrow Biopsy. Nine years post-surgery, there are no signs of recurrence of the lesion. Given the rarity of LCH, we strongly recommend protocolization of treatment. 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.

Bone scintigraphy showed no other lesions. CT may be useful to define the rnfermedad of the process.

LCH has a slight predilection for males and generally appears in childhood 1,4,6,8. Initial stage lesions tend to be very cellular, whereas in more advanced stages, a higher degree of fibrosis is present 6.