Abn Of Dermal Fibrous And Elastic Tissue Flashcards
Fibrillar collagens
I, II, III, V, XI
Most abundant collagen in skin
I
Type III collagen
Fetal skin and blood vessels
Up to 40% in inflamed skin
Fiber associated collagens
VIII, IX, XIV
Found on the surface of type I and II collagen and act as flexible spacers btw fibrils
Angiotensin II type 1 receptor
Stimulation increases collagen production and inhibits degradation.
Type 2 has reverse effect
EPS prognosis
Spontaneous recovery from 6 mo to 5 yrs with scarring
More persistent in downs
EPS reported with
Vasoocclusive dz and stroke
RPC prognosis
Reaches max size in 4 weeks then regresses over 6 to 8 weeks
PXE
Lax and redundant skin
Unchallenged comedones and milia en plaque
Exaggerated NL folds and mental crease in pt under 30
Angiostatin streaks before skin
Skin + angiod streaks = gronblad strandberg syndrome
Angiod streaks
EDS, pagets, sickle cell
Drusen like spots
PXE, show increased fluorescence vs age related drusen
EDS oral manifestations
Supernumerary teeth and odontogenic keratocysts
Spheroids in EDS
Ca from fat necrosis
Aortic root dilation seen in 20% of EDS
Ectopia lentis MC in marfans
If cysteine substitution in fibrillin 1 gene
Homocystenuria
Increased homo and methionine
Decrease cysteine
Found in homo and not marfans
Osteoporosis and a./ v. Thrombosis
Downward lens
Diet recommended for homocysteinuria
Methionine free with betaine supp
Avoidance of alfalfa and bean sprouts
Vit C ameliorates endothelial dysfunction
Cutis Laxa names
Dermatomegaly
Dermatolysis chalazoderma
Pachydermatocele
Recessive form MC and more severe with internal inv
AD cutis laxa
Elastin gene
Nonfamilial cutis laxa cases
Seen in urticaria, lupus, glomerulonephritis, plasma cell dyscrasia, systemic amyloid
Isolated acral dz associated with myeloma and RA
De barsey
Severe cutis laxa, MR, growth retardation, joint laxity ocular and skeletal dz
Blepharochalasis
Lax eyelid skin, usually BL
Lack of elastic fibers and IgA deposits
Ascher syndrome
Blepharochalasis and progressive enlargement of the upper lip (double lip)
Tx is surgical
MC location for anetoderma
Shoulders, upper arms, thighs
Anetoderma associations
Up to 1/2 have lupus, antiphospholipid ab, graves, scleroderma, low Cā, hypergamma, HIV
Anetoderma of prematurity
Related to pressure, adhesives, changes in flow of ions or water under leads, intrauterine borrheliosis
Striae
Decreased upper dermal collagen, lie parallel to epi
Increases elastin fibers
Linear focal elastosis
Males
Increased elastic fibers
Fig 25.9
OI
Lobstein syndrome
Brack syndrome
OI + arthrogryposis