25- Fibrous And Elastic Tissue Flashcards
Collagen type in skin, ligaments and bone
Type 1
Collagen abundant in fetal skin and blood vessels
Type III
Basement membrane is made up of collagen
Type IV and VII
Also known as BP180, this type of collagen contains the target antigens for several immunobullous diseases
Type XVII
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
Elastosis perforans serpiginosa
Inherited disorder involving connective tissue of skin eye and cardiovascular system
Small, circumscribed yellow/cream colored papules on sides of neck and flexures- โchicken skin appearance
Exaggerated nasolabial folds and mental creases
Vascular- hemorrhages
Pesudoxanthoma elasticum (PXE)
Characteristic retinal change in pseudoxanthoma elasticum
-appear earlier than skin changes
Angioid streak
- break in Bruchโs elastic membrane
- reddish brown band around optic disk
Excessive stretchability and fragility of the skin with hyperextensibility of joints
Easy scar formation and formation of fibrous or calcified pseudotumors
Ehlers-Danlos syndrome
This sign is seen in Ehlers-Danlos patients when they can touch the tip of the nose with their tongue
Gorlinโs sign
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
Marfan syndrome
Cardio disease seen in Marfan
Ascending aortic aneurysm
Mitral valve prolapse
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
Homocystinuria
Inelastic loose, redundant skin
drooping skin produces bloodhound like facies- around eyelid, cheeks and neck
Abdomen- large pendulous folds
Cutis laxa (generalized elastolysis)
Localized loss of elastic tissue from herniation of subcutaneous tissue
Lesions protrude on skin and have less resistance than surrounding skin โbutton hole signโ
May be shiny, white and crinkly
Shoulders, upper arms and thighs
Anetoderma ( macular atrophy)
Up to half of anetoderma parients have an accompanying abnormality such as
Lupus Antiphospholipid antibodies Graves Sceroderma Hypocomplementemia Hypergammaglobulinemia Autoimmune hemolysis HIV