Developmental and Immune-mediated Mucocutaneous Conditions - part IV Flashcards
T/F: systemic sclerosis is common
false, rare
systemic sclerosis
characterized by inappropriate deposition of dense collagen
etiology of systemic sclerosis
probably immunologically mediated
systemic sclerosis is also termed what?
scleroderma
sclero = hard derma = skin
clinical features of systemic sclerosis
- diffuse, hard, smooth texture of skin
- Raynaud phenomenon
- sclerodactyly
- acro-osteolysis ± ulceration
- “mask-like” face
- atrophy of alae
- mouse facies
- microstomia “purse-string” appearance
- dysphagia with esophageal involvement
- pulmonary, renal, cardiac and GI fibrosis may be seen, with pulmonary hypertension
sclerodactyly
claw-like deformity
acro-osteolysis
resorption terminal phalanges…
destruction of digit tips, including bone
mouse facies
pinched appearance of nose
which sex is 3x more frequently affected by systemic sclerosis?
women 3x more than men
Raynaud’s phenomenon
discoloraton of fingers and/or toes after exposure to changes in temperature or emotional events causing spasm of blood vessels
T/F: Raynaud’s phenomenon is specific for systemic sclerosis
false, non specific
white discoloration of fingers and/or toes due to Raynaud’s phenomenon is caused by what?
spasm of blood vessels causes diminished blood supply
blue discoloration of fingers and/or toes due to Raynaud’s phenomenon is caused by what?
due to lack of oxygen
red discoloration of fingers and/or toes due to Raynaud’s phenomenon is caused by what?
as blood rushes back in
sclerodactyly is due to what?
collagen deposition, ulceration of fingertips
radiographic features of systemic sclerosis
- diffuse widening of periodontal ligament
- resorption of posterior ramus, condyle, coronoid process or chin
- may see root resorption
resorption of posterior ramus, condyle, coronoid process or chin is seen in what percent of systemic sclerosis pts?
10-20%
histo features of systemic sclerosis
diffuse deposition of collagen throughout the superficial CT
what is suggestive in dx’ing systemic sclerosis?
generally stiffened skin with development of Raynaud’s phenomenon suggestive of dx
how is systemic sclerosis dx’ed?
- skin biopsy
- lab studies
- serologic studies
serologic studies show that pts with systemic sclerosis have autoantibodies directed against what?
Scl-70
serologic studies show what for pts with limited cutaneous systemic sclerosis?
anticentromere antibodies
T/F: tx for systemic sclerosis is difficult and mainly symptomatic
true
several drugs are used to do what in tx’ing symptoms of Raynaud’s phenomenon?
- used to inhibit collagen production
2. increase peripheral blood flow
T/F: steroids are indicated for tx’ing Raynaud’s phenomenon
false, doesn’t seem to help
what is used to tx hypertension especially renal in pts with systemic sclerosis?
ACE inhibitors
tx for systemic sclerosis
- esophageal dilatation
- oral hygiene instruction
- fabricate prostheses with design to accommodate microstomia
death from systemic sclerosis is due to what?
internal organ deposition of collagen
T/F: cardiac involvement in pts with systemic sclerosis has a worse prognosis
true
most deaths from systemic sclerosis is due to what involvement?
pulmonary
what is the 10-year survival rate for limited cutaneous involvement in systemic sclerosis pts?
75-80%
what is the 10-year survival rate for diffuse cutaneous involvement in systemic sclerosis pts?
55-60%
CREST syndrome
Calcinosis cutis Raynaud's phenomenon Esophageal dysfunction Sclerodactyly Telangiectasia
what is a milder variant of systemic sclerosis?
CREST syndrome
who is affected by CREST syndrome?
women, 6-7th decade
calcinotis cutis
subcutaneous nodules are deposition of calcium salts
tx of CREST syndrome if symptomatic
similar to systemic sclerosis
T/F: px of CREST syndrome is worse than systemic sclerosis
false, better than systemic sclerosis
what is the 6-year survival rate for pts with CREST syndrome?
80%
what is the 12-year survival rate for pts with CREST syndrome?
50%
