Description
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Explanation & Answer
Attached.
Scleroderma
Scleroderma
derived from greek and means
hard skin
Affects about 250/million =
7500 Canadians
Affects mainly women in the
prime of their life (women:men ratio 35:1, onset between 30-50 years)
SYSTEMIC SCLEROSIS OR
SCLERODERMA
A generalized disorder of connective
tissue characterized clinically by:
thickening and fibrosis of the
skin (scleroderma)
distinctive forms of
involvement of internal organs,
notably the heart, lungs,
kidneys, and gastrointestinal
tract.
ACR CRITERIA
Sclerodermatous skin change in any location
proximal to the metacarpophalangeal joints
The presence of two or more of the following
features contributed further as minor criteria:
sclerodactyly
pitting scars of fingertips or loss of digital finger pad
substance
bibasilar pulmonary fibrosis
Raynaud’s
Phenomenon
Skin thickening of systemic sclerosis begins
on the fingers and hands in nearly all
cases. The skin initially appears shiny and
taut and may be erythematous at early
stages.
Phases of skin thickening
Edematous
Causes of this swelling
Basically this is water in the tissues = edema
Binding of water to increased extracellular
connective tissue matrix
Inflammation
Poor lymphatic return
Microvascular injury with fluid extravasation
Indurative Phase
Thick
Shiny
Taught
Tightly adheres to underlying tissue so cannot be easily picked up
Creases disappear
May become hyper- or hypo-pigmented
Hair loss
Decreased sweating
Facial changes
Pinched nose
(“mauskopf”)
Pursed lips
...
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