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1.
Pan Afr Med J ; 33: 293, 2019.
Article in English | MEDLINE | ID: mdl-31692902

ABSTRACT

Erdheim-Chester disease (ECD) is a very rare and aggressive form of non-Langerhans histiocytosis with unclear pathogenesis. Because of the heterogeneity of clinical presentation, diagnosis is often challenging and delayed. Currently, Interferon alpha is the first line treatment that is associated with a better survival. The prognosis is relatively poor, especially in case of neurological and cardiovascular involvement. Herein, we report the case of a 64-year-old Tunisian female patient presenting an aggressive form of ECD revealed by diabetes insipidus and cerebellar ataxia with a diagnosis delay of 4 years. The assessment of disease extent had also shown associated asymptomatic cardiac and bone involvement. Pegylated Interferon alpha was started at high dose allowing disease stabilization. This case illustrates that physicians should be aware of the heterogeneous manifestations of ECD in order to insure an early diagnosis and treatment. Long-term and regular follow-up is crucial because of the risk of disease progression.


Subject(s)
Cerebellar Ataxia/etiology , Diabetes Insipidus/etiology , Erdheim-Chester Disease/diagnosis , Delayed Diagnosis , Erdheim-Chester Disease/complications , Erdheim-Chester Disease/drug therapy , Female , Humans , Interferon-alpha/administration & dosage , Middle Aged , Polyethylene Glycols/chemistry , Prognosis , Tunisia
2.
Intern Med ; 53(18): 2091-4, 2014.
Article in English | MEDLINE | ID: mdl-25224194

ABSTRACT

Diabetic muscle infarction (DMI) is a rare complication of long-standing poorly controlled diabetes mellitus. We herein describe the case of a 56-year-old man with a 10-year history of poorly controlled type 2 diabetes mellitus with multiple microvascular and macrovascular complications who presented with the sudden onset of left thigh pain and swelling. MRI suggested muscle infarction. A muscle biopsy demonstrated coagulation necrosis in the skeletal muscle with inflammation and infarction in the walls of small blood vessels. Physicians should consider DMI in the differential diagnosis of patients with diabetes who present with painful, swollen muscles without systemic signs of infection.


Subject(s)
Diabetes Mellitus, Type 2/complications , Hypoglycemic Agents/therapeutic use , Infarction/etiology , Muscle, Skeletal/blood supply , Biopsy , Diabetes Mellitus, Type 2/drug therapy , Diagnosis, Differential , Follow-Up Studies , Humans , Infarction/diagnosis , Magnetic Resonance Imaging , Male , Middle Aged , Thigh
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