Abn Of Dermal Fibrous And Elastic Tissue Flashcards Preview

Dermatology > Abn Of Dermal Fibrous And Elastic Tissue > Flashcards

Flashcards in Abn Of Dermal Fibrous And Elastic Tissue Deck (30):
1

Fibrillar collagens

I, II, III, V, XI

2

Most abundant collagen in skin

I

3

Type III collagen

Fetal skin and blood vessels

Up to 40% in inflamed skin

4

Fiber associated collagens

VIII, IX, XIV

Found on the surface of type I and II collagen and act as flexible spacers btw fibrils

5

Angiotensin II type 1 receptor

Stimulation increases collagen production and inhibits degradation.

Type 2 has reverse effect

6

EPS prognosis

Spontaneous recovery from 6 mo to 5 yrs with scarring

More persistent in downs

7

EPS reported with

Vasoocclusive dz and stroke

8

RPC prognosis

Reaches max size in 4 weeks then regresses over 6 to 8 weeks

9

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

10

Angiod streaks

EDS, pagets, sickle cell

11

Drusen like spots

PXE, show increased fluorescence vs age related drusen

12

EDS oral manifestations

Supernumerary teeth and odontogenic keratocysts

13

Spheroids in EDS

Ca from fat necrosis

Aortic root dilation seen in 20% of EDS

14

Ectopia lentis MC in marfans

If cysteine substitution in fibrillin 1 gene

15

Homocystenuria

Increased homo and methionine
Decrease cysteine

16

Found in homo and not marfans

Osteoporosis and a./ v. Thrombosis
Downward lens

17

Diet recommended for homocysteinuria

Methionine free with betaine supp
Avoidance of alfalfa and bean sprouts
Vit C ameliorates endothelial dysfunction

18

Cutis Laxa names

Dermatomegaly
Dermatolysis chalazoderma
Pachydermatocele

Recessive form MC and more severe with internal inv

19

AD cutis laxa

Elastin gene

20

Nonfamilial cutis laxa cases

Seen in urticaria, lupus, glomerulonephritis, plasma cell dyscrasia, systemic amyloid

Isolated acral dz associated with myeloma and RA

21

De barsey

Severe cutis laxa, MR, growth retardation, joint laxity ocular and skeletal dz

22

Blepharochalasis

Lax eyelid skin, usually BL
Lack of elastic fibers and IgA deposits

23

Ascher syndrome

Blepharochalasis and progressive enlargement of the upper lip (double lip)
Tx is surgical

24

MC location for anetoderma

Shoulders, upper arms, thighs

25

Anetoderma associations

Up to 1/2 have lupus, antiphospholipid ab, graves, scleroderma, low C', hypergamma, HIV

26

Anetoderma of prematurity

Related to pressure, adhesives, changes in flow of ions or water under leads, intrauterine borrheliosis

27

Striae

Decreased upper dermal collagen, lie parallel to epi
Increases elastin fibers

28

Linear focal elastosis

Males
Increased elastic fibers
Fig 25.9

29

OI

Lobstein syndrome

30

Brack syndrome

OI + arthrogryposis