Derm in Cardiology Flashcards
(33 cards)
dermatology findings associated with Marfan’s Syndrome
*ectopia lentis (lens dislocation)
*arched palate
*pectus excavatum (sternal abnormalities)
*skin laxity
*scoliosis
*Marfanoid habitus
*hyper-flexibility of wrists & fingers
*striae distensae (stretch marks)
ectopia lentis (lens dislocation) in Marfan’s Syndrome
*upward lens dislocation
*can be seen on dilated eye exam
*risk for glaucoma later in life
arched palate in Marfan’s Syndrome
*highly arched palate in the roof of the mouth
*easily visualized on oral examination
pectus excavatum (sternal abnormalities) in Marfan’s Syndrome
*congenital abnormality of sternum and ribs
*grow inwards
*often most noticeable during puberty
*requires surgical correction
skin laxity in Marfan’s Syndrome
*skin can be more stretchy, especially on extremities
scoliosis in Marfan’s Syndrome
*abnormal curvature of the spine can be a common manifestation of Marfan Syndrome
Marfanoid Habitus in Marfan’s Syndrome
*abnormalities of the long bones
*very tall stature
*long thin arms & legs
characteristic hand findings in Marfan’s Syndrome
*hyper-flexibility of wrists/fingers
*long spidery appearing fingers
striae distensae in Marfan’s Syndrome
*stretch marks
*increased presence of stretch marks on trunk, hips, thighs
cardiac findings in Marfan’s Syndrome
*mitral valve prolapse
*aortic valve insufficiency
*aortic root dilation
*aortic dissection and/or rupture
Marfan’s Syndrome - genetic mutation
*genetic disorder that affects connective tissues
*arises from a mutation of the fibrillin 1 gene (FBN1) on chromosome 15
*inherited in autosomal dominant fashion with variable expressivity
*individuals affected are characteristically tall and thin, with long arms, legs, fingers, and toes
*also associated with cardiac abnormalities
stasis dermatitis
*seen in patients with poor circulation, such as those with diabetes and vascular disease
*woody discoloration to the skin, mostly anterior leg
*skin becomes firm (indurated) and can be sore to touch
*almost always BILATERAL
*can develop blisters within plaques of stasis dermatitis
venous stasis ulcers
*progression of stasis dermatitis
*when small vessel circulation in the legs becomes poor enough, skin can break down
*ulcers develop on legs, usually ANTERIOR
*painful (moderate intensity)
arterial ulcers
*result of occlusion of small arteries that supply skin
*seen in peripheral vascular disease and thromboembolic disease
*VERY PAINFUL
*punched out appearance
*commonly found on medial malleoli or dorsal foot
lyphedema
*swelling of the extremities, usually legs
*caused by dysfunction of lymphatic system
*contributing illnesses: obesity, pregnancy, prior vascular surgery, removal of lymph nodes
*graded based on scale of pitting and rebound
dermatology findings associated with Ehlers-Danlos Syndrome
*blue sclerae
*gorlin skin
*Ehlers-Danlos facies
*easy bruising
*skin laxity
*fish-mouth scars
*joint laxity
*club foot
blue sclerae in Ehlers-Danlos Syndrome
*thinning of the scleral soft tissue allows it to become translucent, giving it a blue hue
*can be seen in Ehlers-Danlos & osteogenesis imperfecta
Gorlin sign in Ehlers-Danlos Syndrome
*ability to touch the tip of the tongue to the nasal tip
*~50% of patients with inherited connective tissue disease such as Ehlers-Danlos can do this
Ehlers-Danlos Syndrome facies
*several distinguishing facial features:
-small ear lobes
-prominent eyes
-narrow nose
-thin lips
-small chin
easy bruising in Ehlers-Danlos Syndrome
*one of the most common features in Ehlers-Danlos Syndrome
*often mistaken for child or domestic abuse
skin laxity in Ehlers-Danlos Syndrome
*skin can be more stretchy, especially on extremities
fish-mouth scars in Ehlers-Danlos Syndrome
*wide atrophic scars
*often overlying knees or elbows
*poor wound healing with dehiscence (separation) of wounds common
joint laxity in Ehlers-Danlos Syndrome
*hyper-mobile joints can lead to early onset arthritis and joint instability
*may require corrective braces for knees and ankles
club foot in Ehlers-Danlos Syndrome
*malposition of the talus (heel bone) of the foot, often accompanying thickening of surrounding ligaments
*can be corrected with orthotics, physical therapy, or surgery if needed
*about 10% of patients with Ehlers-Danlos Syndrome have club foot
