Signs
and Symptoms of Dermatomyositis In childhood the onset is rather acute
and consists of muscle weakness in the hip muscles and upper arm muscles close
to the trunk (proximal muscle weakness). In older patients this weakness is developing in
a slower fashion. There can be a skin rash associated with it. There is also a
muscle ache and tenderness and pain in many joints. Swallowing can
be a problem when the pharynx and esophagus are involved. A fever
and weight loss are some non specific symptoms that are often also present.
As a result of the proximal muscle involvement the patient may find it difficult
to walk upstairs, and if the breathing muscles are involved there might be breathing
problems, particularly when challenged physically. Muscles in hands, feet and
face are usually spared (Ref. 1). In dermatomyositis a typical purplish
discoloration occurs often around the eyes ("heliotrope hue").
This is usually associated with swelling of the connective tissues around the
eyes called "periorbital edema". Similar rashes may appear
in other locations of the body. When the rash clears, there may be a pigmentation
or thinning of the skin (called"atrophy") that can develop. Alternatively
depigmentation, called "vitiligo", can cause a leopard-like
skin appearance.
| Signs and symptoms
of dermatomyositis | | Symptoms
and signs: | Comments: |
| proximal muscle weakness | in
upper arms, thighs, shoulders and neck flexors |
| other muscle weakness | of
larynx muscles causes dysphonia; of breathing muscles causes dyspnea; of pharynx
and esophagus muscles causes swallowing problems |
| skin involvement | multitude
of appearances: purplish discoloration of skin around eyes; leopard skin from
hyperpigmentations, vitiligo (see text) and skin atrophy; subcutaneous skin calcifications
| | polyarthralgia
| multiple joint involvement with joint swelling,
effusions (non deforming) | | Raynaud's
phenomenon | often in patients with simultaneous
other connective tissue diseases (see text) |
| interstitial pneumonitis | lung
involvement with breathing problems and cough |
| gastrointestinal symptoms | more
common in children: ulcerations in stomach or gut can cause blood loss |
Calcifications
of the subcutaneous skin layers tends to happen more frequently in childhood
than in adults and is another diagnostic criterion. In more serious cases
the lungs can be affected with a condition called "interstitial pneumonitis"
where the patinet becomes short of breath and coughs a lot. A referral to a lung
specialist would lead to the diagnosis, which otherwise could be delayed by thinking
that this is just a virus that hangs around. Raynaud's phenomenon is
common.This condition is due to a narrowing or closing off of the small blood
vessels to the toes fingers thus giving rise to chronic skin ulcerations at the
tip of fingers and toes. Some parts of the toes or fingers are even lost
or have to be amputated. This happens more when dermatomyositis occurs simultaneously
in patients with other connective tissue dieseases such as Sjögren's syndrome
or lupus. Gastrointestinal manifestation of dermatomyositis occurs
more often in children where ulcerations of the gastrointestinal lining can lead
to acute blood loss or to perforations with acute peritonitis. In case of bleeding
there often would be vomiting of blood (upper GI bleed) or the passing of black,
tarry stools (melena). In case of an acute perforation, a surgeon would need to
do a surgical exploration and remove the section of gut with the perforating ulcer.
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