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Eosinophilic granuloma.txt
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Eosinophilic granuloma
Signs and symptoms
Clinically, single or multiple lesions are present mostly on the axial skeleton - on the skull, spine, ribs, pelvis and long bones. Specifically, thoracic spine and frontal bone within the skull are the most affected in children. On the other hand, cervical spine and jaw bone are the major spots of EG lesions in adults. Symptoms of EG include stiffness, local pain, edema of surrounding tissues, posture change, and many others, depending on the affected bone.
Lung EG is even less common. Its prevalence has not been published, however, lung EG was already diagnosed in approximately 5% of all lung biotic samples. Such condition may be asymptomatic, but also a non-productive cough, dyspnea, chest pain, fatigue, and spontaneous pneumothorax can occur. Higher risk of lung EG is being associated with smoking.
Diagnosis
Diagnostic options of EG comprise X-ray, CT and MR. Histopathological examination of the bioptic sample is focused on the presence of mononuclear LCs with prominent nuclear grooves (coffee bean shaped nuclei) with addition of eosinophils. Such examination also utilizes CD1 and CD207 (Langerin) staining. Electron microscopy examination of the sample is based on detection of Birbeck granules, specific "tennis racquet" shaped inclusion within cytoplasm of LCs.
Treatment
Single lesions spontaneously regress often, especially in children, thus are followed up in regular intervals. Patients with spinal lesions are immobilized to minimize the pain. More complicated cases with multiple lesions should be treated with corticosteroids, particularly with an intralesional injection of methylprednisolon. Corticosteroids also represent an option for pulmonary EG treatment. Operative treatment includes bone grafting and surgical fixation. In advanced stages of the disease, lung transplantation may be required. High-risk patients with multiple lesions (CNS-risk bones, lungs) may undergo chemotherapy.
Diagnosis
Diagnostic options of EG comprise X-ray, CT and MR. Histopathological examination of the bioptic sample is focused on the presence of mononuclear LCs with prominent nuclear grooves (coffee bean shaped nuclei) with addition of eosinophils. Such examination also utilizes CD1 and CD207 (Langerin) staining. Electron microscopy examination of the sample is based on detection of Birbeck granules, specific "tennis racquet" shaped inclusion within cytoplasm of LCs.
Treatment
Single lesions spontaneously regress often, especially in children, thus are followed up in regular intervals. Patients with spinal lesions are immobilized to minimize the pain. More complicated cases with multiple lesions should be treated with corticosteroids, particularly with an intralesional injection of methylprednisolon. Corticosteroids also represent an option for pulmonary EG treatment. Operative treatment includes bone grafting and surgical fixation. In advanced stages of the disease, lung transplantation may be required. High-risk patients with multiple lesions (CNS-risk bones, lungs) may undergo chemotherapy.
Treatment
Single lesions spontaneously regress often, especially in children, thus are followed up in regular intervals. Patients with spinal lesions are immobilized to minimize the pain. More complicated cases with multiple lesions should be treated with corticosteroids, particularly with an intralesional injection of methylprednisolon. Corticosteroids also represent an option for pulmonary EG treatment. Operative treatment includes bone grafting and surgical fixation. In advanced stages of the disease, lung transplantation may be required. High-risk patients with multiple lesions (CNS-risk bones, lungs) may undergo chemotherapy.