Multiple cutaneous, oropharyngeal and pulmonary nodules
| العنوان: | Multiple cutaneous, oropharyngeal and pulmonary nodules |
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| المؤلفون: | K. Bhargava, B. Moriarty, Sean Whittaker, Eduardo Calonje, O. Veraitch, R. Mallipeddi |
| المصدر: | British Journal of Dermatology. 173:540-543 |
| بيانات النشر: | Oxford University Press (OUP), 2015. |
| سنة النشر: | 2015 |
| مصطلحات موضوعية: | Erdheim-Chester Disease, Pathology, medicine.medical_specialty, Cord, genetic structures, medicine.medical_treatment, Dermatology, Disease, Multimodal Imaging, Skin Diseases, medicine, Humans, Radionuclide Imaging, Pathological, Aged, 80 and over, CD68, business.industry, Cutaneous nodules, Pharyngeal Diseases, medicine.disease, Magnetic Resonance Imaging, Radiation therapy, Histiocytosis, Hoarse voice, Chronic Disease, Multiple Pulmonary Nodules, Female, Bone Diseases, medicine.symptom, Mouth Diseases, Tomography, X-Ray Computed, business |
| الوصف: | Summary Erdheim–Chester disease (ECD) is a rare non-Langerhans cell histiocytosis defined by heterogeneous multiorgan involvement. Due to the rarity of this disease strong evidence-based therapies have yet to be established and prognosis has previously been considered to be poor, with more than half of patients dying within 3 years of initial presentation. We describe an 86-year-old woman with a 34-year history of extensive cutaneous and internal nodules with typical pathological and immunophenotypical (CD68+/CD1a−) features of ECD without evidence of the BRAF V600E mutation. The cosmetic appearance of cutaneous nodules and hoarse voice caused by vocal cord nodules has been managed surgically. More aggressive therapies reported for ECD were trialled for this patient, such as radiotherapy and interferon-α, with no response. This case demonstrates a relatively good prognosis in ECD that has been managed conservatively. |
| تدمد: | 1365-2133 0007-0963 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::a097b21bef0857c426dcad2bd820c0e4 https://doi.org/10.1111/bjd.13708 |
| حقوق: | CLOSED |
| رقم الأكسشن: | edsair.doi.dedup.....a097b21bef0857c426dcad2bd820c0e4 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 13652133 00070963 |
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