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Juvenile Dermstomyositis Kannemirova M.G. Dermstomyositis «Дерматомиозитные очки» -- отечность и эритематозные высыпания с лиловым оттенком в параорбитальной области Heliotrope is often associated with periorbital edema and telangiectasias of the upper eyelids. In the resolution stage, atrophy or dyspigmentation (hypo- or hyperpigmentation) may be apparent. Heliotrope. Subtle erythema and minimal edema involving both upper eyelids, with extension to the lower eyelids. Dermstomyositis • Heliotrope. Confluent macular erythema confined to the upper eyelid, with associated periorbital edema Juvenile Dermstomyositis • Malar and facial erythema and oedema. Acute onset of confluent macular erythema in a periorbital and malar distribution with extension to the chin in a boy with juvenile dermatomyositis. Note the perioral sparing Juvenile Dermstomyositis • Gottron's papules. Discrete erythematous papules overlying the metacarpal and interphalangeal joints in a patient with juvenile dermatomyositis Juvenile Dermstomyositis • Gottron's papules. Erythematous to violaceous raised papules overlying the metacarpal and interphalangeal joints in a patient with juvenile dermatomyositis Juvenile Dermstomyositis • Gottron's papules. Erythematous plaques overlying the elbows in two patients with juvenile dermatomyositis. In some patients, small erythematous plaques may overly the extensor aspects of larger joints, such as the elbows, knees, and medial malleoli. This is considered to be an extended part of the spectrum of Gottron's papules. Juvenile Dermatomyositis • Gottron's papules showing secondary atrophy and telangiectasia in a girl with severe juvenile dermatomyositis. These changes are seen in association with prominent periungual erythema, a cutaneous finding indicative of ongoing disease activity. Juvenile Dermatomyositis • Gottron's papules showing secondary atrophy and telangiectasia Dermatomyositis Gottron's papules on the dark skin seems black. *Г. Готтрон (Heinrich A. Gottron, 1890-1974) немецкий терапевт. Juvenile Dermatomyositis • Subcutaneous Canodules over the elbows with inflammatory changes Juvenile Dermatomyositis • Muscular calcinosis. Juvenile Dermatomyositis • General dystrophy – the outcome of Juvenile Dermatomyositis Medications to help relieve symptoms include: • • • • • • • Prednisolone Methotrexate Mycophenolate (CellCept / Myfortic) immunosuppressant drug Intravenous immunoglobulin Azathioprine Cyclophosphamide Rituximab[8]-is a chimeric monoclonal antibody against the protein CD20, Polyarteritis nodosa American College of Rheumatology criteria: • • • • • • • • • • Weight loss greater than/equal to 4.5 kg. Livedo reticularis (a mottled purplish skin discoloration over the extremities or torso). Testicular pain or tenderness. (occasionally, a site biopsied for diagnosis). Muscle pain, weakness, or leg tenderness. Nerve disease (either single or multiple). Diastolic blood pressure greater than 90mmHg (high blood pressure). Elevated kidney blood tests (BUN greater than 40 mg/dl or creatinine greater than 1.5 mg/dl). Hepatitis B virus tests positive (for surface antigen or antibody). Arteriogram (angiogram) showing the arteries that are dilated (aneurysms) or constricted by the blood vessel inflammation. Biopsy of tissue showing the arteritis (typically inflamed arteries).[6]