25- Fibrous And Elastic Tissue Flashcards

1
Q

Collagen type in skin, ligaments and bone

A

Type 1

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2
Q

Collagen abundant in fetal skin and blood vessels

A

Type III

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3
Q

Basement membrane is made up of collagen

A

Type IV and VII

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4
Q

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

A

Type XVII

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5
Q

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

A

Elastosis perforans serpiginosa

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6
Q

Inherited disorder involving connective tissue of skin eye and cardiovascular system

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

Vascular- hemorrhages

A

Pesudoxanthoma elasticum (PXE)

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7
Q

Characteristic retinal change in pseudoxanthoma elasticum

-appear earlier than skin changes

A

Angioid streak

  • break in Bruchโ€™s elastic membrane
  • reddish brown band around optic disk
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8
Q

Excessive stretchability and fragility of the skin with hyperextensibility of joints

Easy scar formation and formation of fibrous or calcified pseudotumors

A

Ehlers-Danlos syndrome

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9
Q

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

A

Gorlinโ€™s sign

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10
Q

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

A

Marfan syndrome

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11
Q

Cardio disease seen in Marfan

A

Ascending aortic aneurysm

Mitral valve prolapse

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12
Q

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

A

Homocystinuria

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13
Q

Inelastic loose, redundant skin

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

Abdomen- large pendulous folds

A

Cutis laxa (generalized elastolysis)

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14
Q

Localized loss of elastic tissue from herniation of subcutaneous tissue

Lesions protrude on skin and have less resistance than surrounding skin โ€œbutton hole signโ€

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

A

Anetoderma ( macular atrophy)

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15
Q

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

A
Lupus
Antiphospholipid antibodies
Graves
Sceroderma
Hypocomplementemia
Hypergammaglobulinemia
Autoimmune hemolysis
HIV
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16
Q

Depressed lines or bands of thin, reddened skinโ€”later white, smooth, shiny and depressed

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

A

Striae distensae

17
Q

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

Usually results from Borrelia infection

A

Acrodermatitis chronica atrophicans

18
Q

Which type of osteogenesis imperfecta is lethal

A

Type 2

19
Q

Major cause of death in osteogenesis imperfecta

A

Respiratory failure secondary to severe kyphoscoliosis and head trauma

20
Q

Which type of Osteogenesis imperfecta has a normal life span

A

Type I and IV

21
Q

Brittle bone disease that result from defect in collagenous matrix

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

A

Osteogenesis imperfecta