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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:
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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:
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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]