25- Fibrous And Elastic Tissue

Question 1

Collagen type in skin, ligaments and bone

Answer 1

Type 1

Question 2

Collagen abundant in fetal skin and blood vessels

Answer 2

Type III

Question 3

Basement membrane is made up of collagen

Answer 3

Type IV and VII

Question 4

Also known as BP180, this type of collagen contains the target antigens for several immunobullous diseases

Answer 4

Type XVII

Question 5

Chronic papular keratotic eruption in arciform shape on sides of the nape, grouped in a serpiginous or horseshoe arrangement

Spontaneous resolution -6 mos to 5y after onset with atrophic scarring

Most commonly associated with Down syndrome

Answer 5

Elastosis perforans serpiginosa

Question 6

Inherited disorder involving connective tissue of skin eye and cardiovascular system

Small, circumscribed yellow/cream colored papules on sides of neck and flexures- “chicken skin appearance
Exaggerated nasolabial folds and mental creases

Vascular- hemorrhages

Answer 6

Pesudoxanthoma elasticum (PXE)

Question 7

Characteristic retinal change in pseudoxanthoma elasticum

-appear earlier than skin changes

Answer 7

Angioid streak

• break in Bruch’s elastic membrane
• reddish brown band around optic disk

Question 8

Excessive stretchability and fragility of the skin with hyperextensibility of joints

Easy scar formation and formation of fibrous or calcified pseudotumors

Answer 8

Ehlers-Danlos syndrome

Question 9

This sign is seen in Ehlers-Danlos patients when they can touch the tip of the nose with their tongue

Answer 9

Gorlin’s sign

Question 10

Autosomal dominant disorder of connective tissue due to mutation in fibrillin-1 gene

Tall stature, loose jointedness, dolichocephalic skull, high arched palate, arachnodactyly, pes planus, poor muscle tone, deformed ears

Answer 10

Marfan syndrome

Question 11

Cardio disease seen in Marfan

Answer 11

Ascending aortic aneurysm

Mitral valve prolapse

Question 12

Inborn error of metabolism of methionine

Similar to Marfan but with generalized osteoporosis, AV thrombosis and mental retardation

Facial flush- malar areas, violaceous when reclining

Answer 12

Homocystinuria

Question 13

Inelastic loose, redundant skin

drooping skin produces bloodhound like facies- around eyelid, cheeks and neck

Abdomen- large pendulous folds

Answer 13

Cutis laxa (generalized elastolysis)

Question 14

Localized loss of elastic tissue from herniation of subcutaneous tissue

Lesions protrude on skin and have less resistance than surrounding skin “button hole sign”

May be shiny, white and crinkly
Shoulders, upper arms and thighs

Answer 14

Anetoderma ( macular atrophy)

Question 15

Up to half of anetoderma parients have an accompanying abnormality such as

Answer 15

Lupus
Antiphospholipid antibodies
Graves
Sceroderma
Hypocomplementemia
Hypergammaglobulinemia
Autoimmune hemolysis
HIV

Question 16

Depressed lines or bands of thin, reddened skin—later white, smooth, shiny and depressed

Occur in response to changes in weight or muscle mass and skin tension

Answer 16

Striae distensae

Question 17

Diffuse thinning of skin on extremities, sometimes associated with fibrous bands

Usually results from Borrelia infection

Answer 17

Acrodermatitis chronica atrophicans

Question 18

Which type of osteogenesis imperfecta is lethal

Answer 18

Type 2

Question 19

Major cause of death in osteogenesis imperfecta

Answer 19

Respiratory failure secondary to severe kyphoscoliosis and head trauma

Question 20

Which type of Osteogenesis imperfecta has a normal life span

Answer 20

Type I and IV

Question 21

Brittle bone disease that result from defect in collagenous matrix

Fractures occur early, sometimes in utero
Loose jointedness
Blue sclerae
Thin and translucent skin

Answer 21

Osteogenesis imperfecta