Lipid and rare and disease
1Division of Rheumatology, Gulhane School of Medicine, Ankara, 06018 Etlik, Turkey
2Department of Pathology, Gulhane School of Medicine, Ankara, 06018 Etlik, Turkey
Muscular involvement in Behçet’s disease is rare and there are only a few case reports in the literature. The causes of elevated muscle enzymes in a patient with Behcet’s disease are many, including myositis, drug-induced myopathy, metabolic myopathy, and the disease itself. We herein have defined an algorithmic approach to a patient with Behcet’s disease and elevated muscle enzymes and report a case of coexisting of lipid storage myopathy.
Behçet's disease (BD) is a systemic vasculitis characterized by relapsing episodes of oral aphthous ulcers, genital ulcers, skin lesions, and ocular lesions. Other systems including vascular, gastrointestinal, and neurological systems can be affected; BD is more frequent and has more serious clinical course in men . Muscular involvement is rare and there are only a few case reports in the literature [–].
Disorders of lipid metabolism, namely, “lipidoses” or “lipid storage myopathies, ” can cause variable clinical presentations, often-involving skeletal muscle. Although other systems, including cardiac, hepatic, and metabolic, can be involved, most frequent complaints are muscle pain and fatigue, usually induced by exercise, fasting, and infections . Diagnosis is important, since they can cause morbidity and mortality and are potentially treatable conditions. Blood tests (serum creatin kinase (CK), lactate, acylcarnitine profile, and amino acids), urine testing (organic acids and myoglobin), muscle biopsy (light and electron microscopy and biochemical assays), electromyography (EMG), exercise testing, genetic testing, and magnetic resonance spectroscopy (MRS) are helpful in establishing the diagnosis.
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