A fatal presentation of dermatomyositis with facial swelling

Severe macrophage activation syndrome and central nervous system involvement in juvenile dermatomyositis

“…Vascular proliferation is also a key factor in RA patho-genesis. Kuo et al (4) demonstrated that downregula-tion of GRP78 by transcription small interfering RNA (siRNA) increased vascular endothelial growth factor (VEGF)/VEGF receptor 2 (VEGFR2) pathways to suppress human colon cancer tumour growth. VEGF can act as a direct proinflammatory mediator during the pathogenesis of RA, and inhibition of rheumatoid synoviocytes apoptosis, which contributes to synovial hyperplasia.…”

“…Although immunosuppressive agents including cyclophosphamide (2,3), azathioprine (3), mycophenolate mofetil (4), and imatinib (5) have been used to reduce lung inflammation and slow down the clinical course of scleroderma lung disease, the results from recent randomized trials assessing these agents (2)(3)(4)(5) have shown mild benefits with some serious side-effects. IVIg therapy has multiple impacts on immune system dysfunction in autoimmune or inflammatory conditions.…”

Periorbital edema

“…Periorbital edema can be, for example, the forme fruste symptom for dermatomyositis, an uncommon connective tissue disorder [36][37][38][39]. In one study, an 81-year-old man presented with bilateral intractable periorbital edema and not until 6 months later, when the patient had dysphagia was the diagnosis of dermatomyositis considered [40].…”

“…To understand better the more common Spider bite [20,21,63] Dermatographism [59] Hepatitis B [10] Tumor necrosis factor receptor-associated periodic syndrome [60,64] Chagas disease [18] Cutis laxa [65] Trichinellosis [14] Blepharochalasis [57,66,67] Epstein-Barr virus [8,9,68] Eyelid metastasis [69] Ophthalmofilariasis [15] Rosacea [51,52] Pott's puffy tumor [28] Sickle cell orbital infarction [70] Necrotising fasciitis [23] Langerhans cell histiocytosis [71] Ophthalmomyiasis [16] Tolosa-Hunt syndrome [72,73] Blister beetle dermatitis [22] Dermatomyositis [36,39,40,74] Rocky Mountain spotted fever [13] Juvenile dermatomyositis [37,38,75] Amebiasis [19] Sarcoid [76] Leukemia [77] Indeed, by definition, these cases were reportable.…”

A Fatal Presentation of Dermatomyositis

“…[1][2][3][4][5][6] The prevalence of DM is not well-defined, as it is historically grouped together with polymyositis (PM) and inclusion body myositis (IBM) in most epidemiologic studies. 7 Currently classified as an idiopathic inflammatory myopathy (IIM), much of the work in understanding DM has been focused on the muscle pathology that accompanies this disorder.…”