Joint, bone, spine : revue du rhumatisme
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Hereditary recurrent fevers are rare genetic diseases characterized by apparently spontaneous attacks of inflammation. They include familial Mediterranean fever (FMF); tumor necrosis factor (TNF) receptor periodic syndrome (TRAPS); hyperimmunoglobulinemia D syndrome (HIDS); and hereditary periodic fevers related to mutations in the CIAS1 (cold induced autoinflammatory syndrome 1) gene, such as Muckle-Wells syndrome, familial cold urticaria, and CINCA/NOMID (chronic infantile neurological cutaneous and articular/neonatal-onset multisystemic inflammatory disease). Musculoskeletal manifestations are common. ⋯ Among differential diagnoses, infection should be considered routinely. The treatment of the inflammatory attacks is nonspecific. New pathophysiological insights have led to the development of promising maintenance treatments designed to reduce the number and severity of the inflammatory attacks and to diminish the risk of secondary amyloidosis.
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Case Reports
Clinical and pathologic manifestations of necrobiosis lipoidica-like skin involvement in sarcoidosis.
Necrobiosis lipoidica dibeticum (NLD) is a granulomatous skin disease mostly associated with diabetes mellitus. NLD has been reported in patients with other systemic disease. ⋯ The association of NLD-like skin lesion in sarcoidosis is not widely appreciated. The subject of NLD and sarcoidosis is reviewed.
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Thoracic myelopathy secondary to OLF is a rare disease described almost exclusively in Japanese patients. Few series of OLF in South Korean subjects has previously been published. This study is to describe the clinical and radiologic aspects, as well as surgical outcomes in a group of South Korean patients. ⋯ OLF is a rare cause of thoracic myelopathy. The frequency appears to have been rarely reported in South Korean subjects. CT with sagittal reconstructions and MRI are helpful for diagnosis and spinal cord involvement. When neurologic symptoms develop, decompressive laminectomy should be done immediately and the surgical outcome is generally good if hyperintense intramedullary signal changes of spinal cord have not yet developed.