| What is Dermatomyositis?
Dermatomyositis is one of a group of
acquired muscle diseases called inflammatory myopathies. The disease,
which
has a subacute (somewhat short and relatively severe) onset, affects both
children and adults. Females are more often
affected than males. Dermatomyositis is characterized by a rash
accompanying, or more often, preceding muscle
weakness. The rash is described as patchy, bluish-purple discolorations on
the face, neck, shoulders, upper chest,
elbows, knees, knuckles, and back. Some patients may also develop hardened
bumps of calcium deposits under the
skin. The most common symptom is muscle weakness, usually affecting those
muscles that are closest to the trunk
of the body (proximal). Eventually, patients have difficulty rising from a
sitting position, climbing stairs, lifting
objects, or reaching overhead. In some cases, distal muscles (those not
close to the trunk of the body) may be affected
later in the course of the disease. Trouble with swallowing (dysphagia)
may occur. Occasionally, the muscles ache
and are tender to touch. Patients may also feel fatigue and discomfort and
have weight loss or a low-grade fever.
Is there any treatment?
Treatment for dermatomyositis generally
consists of a steroid drug called prednisone. For patients in whom
prednisone is not effective, other immunosuppressants such as azathioprine
and methotrexate may be prescribed.
Recently, a drug called intravenous immunoglobulin was shown to be
effective and safe in the treatment of the
disease. Physical therapy is usually recommended to preserve muscle
function and avoid muscle atrophy.
What is the prognosis?
Most cases of dermatomyositis respond to
therapy. The disease is usually more severe and resistant to therapy in
patients with cardiac or pulmonary problems.
What research is being done?
The NINDS conducts and supports a broad
range of research on neuromuscular disorders such as inflammatory
myopathies. The goals of these studies are to conduct therapeutic trials
to increase understanding of the disorders
and, ultimately, to find ways to prevent and cure them. |