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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At the centre of the lytic process a button sequestrum may appear.
Langerhans’ cell histiocytosis
Ve Clin North Am. Plain radiographs show LCH bony lesions as non-calcified, lytic areas without peripheral sclerosis. 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. Report of three cases. Hicks J, Flaitz CM.
Scintigraphy is useful for evaluating extent of the disease and for monitoring its evolution 7. Proceedings of the Pathological Society of Philadelphia, When lymphatic nodes are involved, the most adequate treatment is excision of affected nodes.
Tumors of the Head and Neck in children. Langerhans cell histiocytosis in adults.
Figure 1 Plain radiographs Figure 1a. Langerhans cells are oval or rounded in shape, pale, and have a predominantly eosinophilic cytoplasm 2,8. Xchuller cell histiocytosis may also affect the pituitary gland which makes hormones that control other glands and many body functions, especially growth.
In the maxillofacial area, skin affectation may appear as a papular rash; scalp involvement has a seborrhoea-like presentation. 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.
An orthopantomogram showed extensive bone loss with respect to previous radiographs, involving right maxillary mesial surface of 17 to distal surface of 12 Fig. Smoking cessation may lead to reversal of changes or evolution to pulmonary hannd and pulmonary hypertension. In Cases 1 and 2, there has been no evidence of local relapse in 9 years and 1 year of disease evolution, respectively. The radiological image of “floating teeth” is typical of maxillary and mandibular involvement 2,3,5,11,14, Letterer-Siwe disease acute disseminated diseaseHand-Schuller Christian disease multifocal or uni-focal diseaseand Eosinophilic granuloma usually uni-focal disease Table.
Biopsy revealed the diagnosis of LCH.
enfermedad de Hand-Schüller-Christian
Definition CSP group of disorders of histiocyte proliferation which includes Letterer-Siwe disease; Hand-Schueller-Christian syndrome; and eosinophilic granuloma; Langerhans cells are components of the lesions.
Contributions to medical and biological research, dedicated to Sir William Osler. Diagnosis Skin biopsy Bone Marrow Biopsy.
CT of the pelvis soft tissue window: Maxillofacial manifestations of Langerhans cell histiocytosis: 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 all three cases presented in this report, the lesions were surgically resected.
Plain radiographs of the skull showed large lesions, giving the appearance of “geographic skull” and “floating teeth”. 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, Lateral view of the skull: Langerhans cell histiocytosis in the maxillofacial area in adults. TavernarakiFeb Trans Am Ophthalmol Soc ; The classic triad is exophthalmos, diabetes insipidus and skull lesions. Imaging of soft tissue tumors.
Arch Otolaryngol Head Neck Surg ; Hematology and Oncology – Histiocytosis Pages. The differential diagnosis includes: