Dermatomyositis

Dermatomyositis is one of a group of acquired muscle diseases called inflammatory

myopathies (disorder of muscle tissue or muscles), which are characterized by chronic
muscle inflammation accompanied by muscle weakness. The cardinal symptom is a
skin rash that precedes or accompanies progressive muscle weakness.
Causes:
The cause of this disorder is unknown. It is theorized that an autoimmune reaction
(reactions caused by an immune response against the body's own tissues) or a viral
infection of the skeletal muscle may cause the disease. In addition, some doctors think
certain people may have a genetic susceptibility to the disease
Symptoms:
You may also have muscle weakness that gets worse over weeks or months. This
muscle weakness usually starts in your neck, arms, or hips and can be felt on both
sides of your body.

Other symptoms you might experience are:

 muscle pain
 muscle tenderness
 problems swallowing
 lung problems
 hard calcium deposits underneath the skin, which is mostly seen in children
 fatigue
 unintentional weight loss
 fever
Cutaneous Manifestations of Dermatomyositis
 Calcinosis cutis—calcification of the skin
 Cutaneous vasculitis —persistent skin lesions, tender palpable purpura papules,
ulcers, infarct, and possible digital gangrene caused by small- and medium-
vessel vasculitis (inflammation of the blood vessels)

 Dystrophic cuticles
 Facial swelling
  Gottron papules—violaceous erythematous papules overlying the dorsal
interphalangeal and metacarpophalangeal joints, elbow, or patella
 Heliotrope discoloration of the eyelids
 Macular erythema of the face and forehead

 Mechanics’ hands—roughening and dirty hyperpigmentation of the

dermaglyphics (lines on the palms) or the hands associated with fissuring
(creation of cracks, clefts, or crevices in the skin)
 Periungual telangiectasias—dilated blood vessels of the gingival and toenail folds
giving a red, striated appearance
 Scaly alopecia—loss of hair, with scaly plaques on the scalp

 Shawl sign—macular erythema of the posterior shoulders and neck

Diagnosis:
 An MRI to look for abnormal muscles
 An electromyography (EMG) to record electrical impulses that control your
muscles
 A blood analysis to check your levels of muscle enzymes and autoantibodies,
which are antibodies that attack normal cells
 A muscle biopsy to look for inflammation and other problems associated with the
disease in a sample of muscle tissue
 A skin biopsy to look for changes caused by the disease in a skin sample
Possible complications of dermatomyositis
The muscle weakness and skin problems linked with dermatomyositis can cause a
number of problems. Some common complications are:
 skin ulcers
 gastric ulcers
 difficulty breathing
 lung infections
 problems swallowing
 malnutrition
 weight loss
Associated conditions
Dermatomyositis may cause other conditions or put you at higher risk of developing
them, including:
  Raynaud's phenomenon. This condition causes your fingers, toes, cheeks, nose
and ears to turn pale when exposed to cold temperatures.
 Other connective tissue diseases. Other conditions, such as lupus, rheumatoid
arthritis, scleroderma and Sjogren's syndrome, can occur with dermatomyositis
(overlap syndromes).
 Cardiovascular disease. Dermatomyositis can cause heart muscle inflammation
(myocarditis). In a small number of people who have dermatomyositis,
congestive heart failure and heart arrhythmias develop.
 Lung disease. Interstitial lung disease can occur with dermatomyositis. Interstitial
lung disease refers to a group of disorders that cause scarring (fibrosis) of lung
tissue, making the lungs stiff and inelastic. Signs and symptoms include a dry
cough and shortness of breath.
 Cancer. Dermatomyositis in adults has been linked to an increased likelihood of
developing cancer, particularly of the cervix, lungs, pancreas, breasts, ovaries
and gastrointestinal tract. Risk of cancer increases with age, although it appears
to level off three years or so after a diagnosis of dermatomyositis.
Dermatomyositis can also develop after you receive a diagnosis of cancer.
Treatment:
There's no cure for dermatomyositis, but treatment can improve your skin and your
muscle strength and function.

Medications
Medications used to treat dermatomyositis include:
Corticosteroids. Drugs such as prednisone can control dermatomyositis symptoms
quickly. But prolonged use can have serious side effects.
Corticosteroid-sparing agents. When used with a corticosteroid, these drugs can
decrease the dose and side effects of the corticosteroid. The two most common
medications for dermatomyositis are azathioprine (Azasan, Imuran) and methotrexate
(Trexall).
Rituximab (Rituxan). More commonly used to treat rheumatoid arthritis, rituximab is an
option if initial therapies don't adequately control your symptoms.
Antimalarial medications. For a persistent rash, your doctor might prescribe an
antimalarial medication, such as hydroxychloroquine (Plaquenil).
Sunscreens. Protecting your skin from sun exposure by applying sunscreen and
wearing protective clothing and hats is important for managing the rash of
dermatomyositis.
Other treatments that can help with muscle problems brought on by dermatomyositis
include:

 Heat therapy
 Exercise
 Orthotics
 Devices that help you stand and move
 Rest
Surgical and other procedures
 Intravenous immunoglobulin (IVIg). IVIg is a purified blood product that contains
healthy antibodies from thousands of blood donors. These antibodies can block
the damaging antibodies that attack muscle and skin in dermatomyositis. Given
as an infusion through a vein, IVIg treatments are expensive and might need to
be repeated regularly for the effects to continue.
 Surgery. Surgery might be an option to remove painful calcium deposits and
prevent recurrent skin infections.
Disability:
Those with polymyositis and dermatomyositis often have significant physical
impairments. For instance, muscle weakness and loss, calcium deposits, pain, fatigue,
and shortness of breath can all affect an individual’s ability to stand and walk or to carry
or lift objects in the workplace. In fact, some individuals need to use a wheelchair
because of this. The inability to sit for a long period of time would affect one’s ability to
do a desk job. The limitations on arm movement and fine motor skills will also further
limit one's ability to do any job.