Guang-li Shen | PubMed Health

PubMed2006

[Immunopathological changes of micro-vessels in dermatomyositis].

OBJECTIVE: To investigate the immunopathological changes of micro-vessels in dermatomyositis. METHODS: Twelve patients with dermatomyositis, 5 males and 7 females, aged 40.8 (6 - 72), underwent muscle biopsy of biceps muscle of arm (n = 11) or deltoid muscle (n = 1). The specimens underwent routine histological, enzyme histochemical, and immunohistochemical staining and microscopy. Ten biopsy specimens from patients with other diseases were used as controls. RESULTS: Perifascicular atrophy of muscular fibers and inflammatory infiltration in perimysium were seen in all patients with dermatomyositis. Non-specific esterase staining showed deep staining of capillaries and micro vascular endothelium among the muscular fibers. Immunohistochemistry showed remarkable reduction of capillaries positive in von Willebrand factor (vWF), thrombomodulin (TM), and endothelial cell nitric oxide synthase (eNOS) in the perifascicular region, and low expression of .eNOS and TM in the microvascular endothelium in the perimysium. CONCLUSION: The vascular lesions of dermatomyositis are located not only in capillaries, but also in other microvessels. Lower expression of TM and eNOS in vascular endothelium suggests the reduction of anticoagulation and vasodilation functions of vascular endothelium. Dermatomyositis is an inflammatory vascular endothelial disease.

Adolescent↗

PubMed2005

[Diffuse muscular calcification with subcutaneous cysts].

OBJECTIVE: Diffuse muscular calcification was rare myopathological change due to abnormal metabolism of calcium, which was mainly found in dermatomyositis and myositis ossificans progressiva. Here we reported a case of diffuse muscular calcification that clinically mimicked myositis ossificans progressiva. The disease might be a new type of congenital calcium metabolic disease. METHODS: A 15-year-old girl developed subcutaneous cysts in the wrist and ankle when she was 1 year old. At the age of 9, she developed recurrent fever with myalgia, fatigue and diffuse muscular calcification. It was difficult for her to squat, run or walk. Protuberance presented in the subcutaneous tissue of her trunk. Some nodules ruptured with outflow of chalky material. ESR, ENA, RF, CRP, PTH, CK were in normal limits. EMG was unremarkable. X-ray confirmed diffuse calcification in the muscle and subcutaneous tissues. Biceps muscle biopsy was performed. RESULTS: Numerous inflammatory cells infiltrated around vessels in the perimyosium with perifascicular muscle fiber atrophy and degeneration. Many RRF and SDH positive fibers were also observed. EM showed tubular reticular inclusions in vascular endothelium. CONCLUSION: Diffuse muscular calcification indicated existence of systemic calcium metabolic abnormality. As the clinical symptoms and distribution pattern of calcification were different from dermatomyositis with subcutaneous calcification and myositis ossificans progressiva, our case might be a new type of disease. The microvascular changes might result in the lesion of muscle fibers.

Adolescent↗