April 13, 2020   |   by admin

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: Dousar Moogukinos
Country: Haiti
Language: English (Spanish)
Genre: Literature
Published (Last): 17 August 2007
Pages: 466
PDF File Size: 5.9 Mb
ePub File Size: 4.27 Mb
ISBN: 850-9-36734-750-8
Downloads: 60719
Price: Free* [*Free Regsitration Required]
Uploader: Mikatilar

Ann Otol Laryngol ; Eur J Cancer ; Sutton D ed Textbook of Radiology and imaging.

TI-weighted image with contrast: LCH has a slight predilection for males and generally appears in childhood 1,4,6,8. It mainly affects children, but adult cases also occur, with an incidence rate of one to two per million.

The incidence of the disease in the adult population is from one to two cases per million inhabitants, ranging in presentation from 15 to 91 years, with mean age of diagnosis 35 years 4, 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, Subsequently, two additional surgeries were undertaken due to the appearance of new lesions in the right malar bone, right and left hemimaxillae, and left mandibular body Fig.

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. For example, the rate of recurrence of the disease falls markedly with 6-month treatments of prednisone and vinblastine. Hematology and Oncology – Histiocytosis Pages. In the long bones, lesions usually arise in the diaphysis or metaphysis and are centred in the medullary cavity causing endosteal scalloping and eventually cortical penetration.


In the past, LCH was subdivided into three categories: Figure 1 Plain radiographs Figure 1a. Back Links pages that link to this page. Periodontitis asociada a enfermedad sistemica en el paciente pediatrico. The lesions may be well defined or poorly defined with or without associated sclerotic borders.

When lymphatic nodes are involved, the most adequate treatment is excision of affected nodes. A neoplastic proliferation of Langerhans cells which contain Birbeck granules by ultrastructural examination. There was no evidence of periosteal reaction or marginal sclerosis. Report of three cases. Defects in membranous bones, exophthalmos, and diabetes insipidus; an unusual syndrome of dyspituitarism.

A year-old female was referred to the Dept. The radiological image of “floating teeth” is typical of maxillary and mandibular involvement 2,3,5,11,14, InLichtenstein observed cytoplasmic bodies, known as X bodies, within histiocytes from tissues of patients suffering from what schhuller previously considered distinct clinical disorders: Resolution of cutaneous lesions of histiocytosis X by intralesion injections of interferon-beta.

Hematology and Oncology Chapters.

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

Int J Dermatol ; Once the condition has been diagnosed, adequate workup to determine the extent of the disease is mandatory 8, Large lesions give the appearance of “geographic skull”.

However, in Case 3, due to the rapid and multiple bony relapses in the maxillofacial area following surgeries, we administered chemotherapy consisting of vinblastine and methotrexate, with favourable results.

Started inthis collection dw contains interlinked topic pages divided into a tree of 31 specialty books and chapters. The clinical course is generally related to the number of organs affected at presentation. Given the rarity of LCH, we strongly recommend protocolization of treatment. A variable amount of periosteal reaction can be present, ranging from a thick solid type to lamellated. In spite of being considered a disease of sporadic appearance, some authors have postulated the existence of a familiar predisposition 4,8, Four years following surgery, the patient was diagnosed with hypogonadotropic hypogonadism and diabetes insipidus.


Content is updated monthly with systematic literature reviews and conferences. There are no controlled trials proposing an optimal treatment protocol for LCH. In the majority of refractory enfermexad 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.

Manifestation of LCH may take various forms.

Infobox medical condition new All stub articles. There is involvement of multiple sites in one organ system, most frequently the bone. Initial stage lesions tend to be very cellular, whereas in more advanced stages, a higher degree of fibrosis is present 6. Chdistian advocate surgical treatment of the lesion by means of curettage or biopsy 4,6,7,11,12,14or intralesional injection of corticosteroids 2,6,11,12, Maxillofacial manifestations of Langerhans cell histiocytosis: 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.

In all three cases presented in this report, the lesions were surgically resected.

There was a problem providing the content you requested

The lesion was elastic to the touch, painful, and enfermwdad normal upper eyelid movement Fig. Nine years post-surgery, there are no signs of recurrence of the lesion. Quintessence Publishing Co, Inc; After adequate workup to determine the extent of the disease, no other lesions were encountered. The lesion was resected once again, and the histopathological report revealed LCH.