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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Disclaimer:

This outline is only a teaching aid to patients and should stimulate you to ask the right questions when seeing your doctor. However, the responsibility of treatment stays in the hands of your doctor and you.

References:

1.The Merck Manual, 7th edition, by M. H. Beers et al., Whitehouse Station, N.J., 1999. Chapter 50.

2. WA Schmidt et al. Clin Rheumatol 2000;19(5):371-377.

3. A Sauty et al. Eur Respir J 1997 Dec;10(12):2907-2912.

4. R Queiro-Silva et al. J Rheumatol 2001 Jun;28(6):1401-1404.

5. J Wada et al. Clin Exp Immunol 2001 May;124(2):282-289.

Last Modified: Jan. 26, 2008