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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Plain radiographs of the skull showed large lesions, giving the appearance of “geographic skull” and “floating teeth”. Radiographs of the femur and pelvis showed multiple lytic lesions with ill-defined borders, including one lesion of the acetabulum. Computer tomography of the pelvis and femur showed lytic leasions with disruption of the cortex and small soft tissue mass. MRI of the hips and femur showed isotense lesions on T1-weighted images, which showed enhacement with gadolinium, and high signal areas of bone replacement on T2-weighted images.
Discussion Hand-Schuller-Christian disease is a chronic disseminated form of histiocytosis X, in which idiopathic non-neoplastic proliferation of histiocytes occurs. It may involve any bone, but preferred sites are the skull, mandible, spine and long bones. The lesions may be well defined or poorly defined with or without associated sclerotic borders.
A variable amount of periosteal reaction can be present, ranging from a thick solid type to lamellated. In the skull, lesions usually have sharply defined borders with uneven involvement of the inner and outer table.
At the centre of the lytic process a button sequestrum may appear. Large lesions give the appearance of “geographic skull”. In the mandible and maxilla, bone destruction may produce the appearance of “floating teeth”.
In the spine, typically, there is lysis of affected vertebrae leading to progressive collapse vertebra plana with preservation of adjacent xchuller space, which usually allows distinction from infection. In the long bones, lesions usually arise in the diaphysis or metaphysis and are centred in christlan medullary cavity causing endosteal scalloping and eventually cortical penetration.
The lesions may also appear as permeative lesions with ill-defined borders and periosteal reaction. Bone scintigraphy is of limited usefulness.
enfermedad de Hand-Schüller-Christian
CT may be useful to define the extent of the process. On MRI examination, T1-weighted images reveal a lesion isointense to adjacent tissue and T2-weighted images reveal high signal areas of marrow replacement. The differential diagnosis includes: Long bones aggressive form: Long bones less aggressive form: Finally, the disease may involve the liver, spleen, lymph nodes, skin, and lungs. The classic triad is exophthalmos, diabetes insipidus and skull lesions.
Langerhans’ cell histiocytosis
Imaging of soft tissue tumors. Radiol Clin North Am. Fundamentals of skeletal radiology. Churchill Livingstone, London TavernarakiFeb En face view of the mandible: Plain radiograph of the femur.
CT of the pelvis osseous window: CT of the pelvis soft tissue window: CT of the femur osseous window. CT of the femur soft tissue window. TI-weighted image with contrast: Search Cases Advanced Search. Case Hand-Schuller-Christian disease Author s.
Clinical History The patient presented with local pain, swellingtenderness of the thigh, and loose teeth. There were not any systemic signs or symptoms.
Imaging Findings The patient presented with local pain, swellingtenderness of the thigh, and loose teeth. Final Diagnosis Hand-Schuller-Christian disease. Sutton D ed Textbook of Radiology and imaging.
Figure 1 Plain radiographs Figure 1a. Lateral view of the skull: