Pediatric emergency medicine trisk 2966 2966
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The onset of JDM is often insidious, with aches and pains in the limbs, malaise, lowgrade fever, and edema of the hands, feet, and eyelids. There may be a diffuse and
nonspecific rash. This prodromal stage evolves into the acute phase, when the
characteristic features of JDM become evident. Classical skin manifestations include a
violaceous heliotrope rash in the periorbital region and occasionally on the forehead;
dusky red or atrophic lesions over the extensor aspects of the knees, elbows, and
knuckles (Gottron papules); and periungual erythema ( Fig. 101.5 ). Skin findings may
precede or follow the onset of muscle weakness. Ulcerative skin lesions and anasarca
are rare presenting manifestations of JDM associated with a particularly severe disease
course.
Muscular involvement in JDM is characterized by symmetric proximal muscle
weakness that may be accompanied by pain and tenderness. Strength of the anterior
neck flexors and abdominal muscles is particularly affected, while facial muscles are
spared. The disease may progress to involve the muscles of the palate and pharynx,
resulting in regurgitation, nasal voice, and aspiration. Weakness of the respiratory
muscles can lead to a poor cough, pneumonia, and respiratory failure. Risk of GI
hemorrhage and perforation are increased at this stage.
The clinical course of JDM is variable; however, with the newer approaches to
treatment, disease manifestations may be controlled within a few months in the vast
majority of patients. Children with ongoing muscle inflammation for more than 6 to 12
months are at risk of developing late complications of JDM. These include pronounced
muscle wasting, contractures, lipodystrophy, and pigmentary changes of the skin. The
rash over the extremities often becomes dry, scaly, and atrophic. Subcutaneous
calcifications historically have occurred in up to 30% of children during this phase,
although early aggressive treatment of inflammation dramatically reduces this rate.
Calcifications are most typically discrete nodules around large joints, but at times they
may take the form of a diffuse encasement of the soft tissues, known as calcinosis
universalis. Occasionally, children pass through the early stages insidiously and come
to the attention of physicians only when they develop contractures and calcinosis.
Clinical Assessment
Although skin and striated muscle are the primary targets of the inflammatory process
in JDM, typically other organ systems are also involved. Up to one-third of children
develop arthritis, which may be present at diagnosis or may develop months into the
disease process. The arthritis of JDM is generally nonerosive and often improves as the
primary disease is treated, although some children require specific therapy for arthritis.
Neurologic manifestations of JDM are extremely rare, but peripheral polyneuropathy,
seizures, psychosis, and one case of suspected brainstem vasculopathy have been
reported.
