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DERMATOMYOSITIS -Predilection sites: nape of the neck, -F>M; common in blacks; relatively rare -Prednisone: the mainstay
upper chest (V) pattern, and upper back, of acute treatment (dooses
neck, and shoulder (shawl) pattern -Evolve through multiple sequential phases: beginning with
1) A genetically determined susceptibility phase 1mg/kg/day until the
2) An induction phase triggered by an environmental severity decreases and
stimulus (eg. Ultraviolet light, infection) muscle enzymes are
involving loss of tolerance to self-antigens in skin almost normal)
and skeletal muscle
3) An autoimmune expansion phase -Methotrexate and
4) An injury phase involving multiple immunologic mycophenolate mofetil
effector mechanisms are used as steroid-sparing
-Edema and pink violet discoloration are agents
often signs of inflammation in the -Characterized by inflammatory myositis and skin
underlying striated orbicularis oculi disease -Daily use of sunscreen
muscle (Heliotrope rash) with high SPF
-Variant:
a) Amyopathic DM: subclinical or absent -Antimalarials, such as
myopathy hydroxychloroquin are
-Telangiectatic vessels often become b) Polymyositis PM: muscle involvement without useful in abating the
prominent in the proximal nailfolds skin changes eruption of DM
Muscle involvement
Skin involvement commonly precedes muscle
involvement but some patients have typical skin
findings of DM but never develop clinically
apparent muscle involvement
Muscle weakness is seen symmetrically involving
the shoulder girdle, pelvic regions, and hands
Difficulty in lifting even the lightest object,
unable to raise their arms to comb their hair and
rising from a chair may be impossible without
“pushing off” with the arms
Pain in the legs when standing barefoot or of
being unable to climb stairs
Difficulty in swallowing, talking, and breathing
Some patients with severe diaphragmatic disease
require mechanical ventilation and cardiac failure
may be present in the terminal phase of the
disease
Calcium deposits in the skin and muscle: 50% in
children with DM
Calcification is related to duration of disease
activity and severity
Calcinosis of the dermis, subcutaneous tissue, and
muscle occur mostly on the upper half of the body
around the shoulder girdle, elbows, and hands
Laboratories
Elevated serum levels of creatine kinase
Other indicators of active muscle disease:
aldolase, lactic dehydrogenase and transaminases
Laboratories: Leukocytosis, anemia with low
serum iron and an increased ESR
Positive ANA test are seen in 60-80%; 35-40%
have myositis specific antibodies