1. Dermatomyositis and Whipple's disease.
- Author
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Helliwell TR, Appleton RE, Mapstone NC, Davidson J, and Walsh KP
- Subjects
- Actinobacteria genetics, Adolescent, Cardiac Output, Low etiology, Dermatomyositis diagnosis, Dermatomyositis pathology, Humans, Immunohistochemistry, Male, Muscle, Skeletal metabolism, Muscle, Skeletal pathology, Muscle, Skeletal ultrastructure, RNA, Bacterial analysis, RNA, Ribosomal, 16S analysis, Tachycardia, Ventricular etiology, Whipple Disease diagnosis, Whipple Disease genetics, Whipple Disease microbiology, Dermatomyositis etiology, Whipple Disease complications
- Abstract
A 14-year-old boy presented with a 3-year history of a skin rash typical of juvenile dermatomyositis, and a 2-month history of mild proximal weakness, myalgia, and weight loss. A quadriceps biopsy showed perifascicular fibre atrophy, focal necrosis and regeneration, immunohistochemical labelling for HLA-1 on the surface of the fibres, and focal C5-9 deposition in capillaries. Macrophages with diastase-resistant, PAS-positive cytoplasm were present. Ultrastructural studies showed electron dense and membranous debris. The patient's symptoms responded to intravenous immunoglobulin and oral prednisolone. Four months after discontinuing prednisolone, the patient developed cardiac failure, ventricular tachycardia, and a recurrence of his rash. The 16S ribosomal RNA specific for Tropheryma whippelii was identified by polymerase chain reaction (PCR) analysis in skeletal and cardiac muscle. The myalgia and skin rash responded to prednisolone and oral co-trimoxazole, and the tachycardia is controlled by oral verapamil. This patient appears to have a novel association of juvenile dermatomyositis and Whipple's disease.
- Published
- 2000
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