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)
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1
Q

Fibrillar collagens

A

I, II, III, V, XI

2
Q

Most abundant collagen in skin

A

I

3
Q

Type III collagen

A

Fetal skin and blood vessels

Up to 40% in inflamed skin

4
Q

Fiber associated collagens

A

VIII, IX, XIV

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

5
Q

Angiotensin II type 1 receptor

A

Stimulation increases collagen production and inhibits degradation.

Type 2 has reverse effect

6
Q

EPS prognosis

A

Spontaneous recovery from 6 mo to 5 yrs with scarring

More persistent in downs

7
Q

EPS reported with

A

Vasoocclusive dz and stroke

8
Q

RPC prognosis

A

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

9
Q

PXE

A

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
Q

Angiod streaks

A

EDS, pagets, sickle cell

11
Q

Drusen like spots

A

PXE, show increased fluorescence vs age related drusen

12
Q

EDS oral manifestations

A

Supernumerary teeth and odontogenic keratocysts

13
Q

Spheroids in EDS

A

Ca from fat necrosis

Aortic root dilation seen in 20% of EDS

14
Q

Ectopia lentis MC in marfans

A

If cysteine substitution in fibrillin 1 gene

15
Q

Homocystenuria

A

Increased homo and methionine

Decrease cysteine

16
Q

Found in homo and not marfans

A

Osteoporosis and a./ v. Thrombosis

Downward lens

17
Q

Diet recommended for homocysteinuria

A

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

18
Q

Cutis Laxa names

A

Dermatomegaly
Dermatolysis chalazoderma
Pachydermatocele

Recessive form MC and more severe with internal inv

19
Q

AD cutis laxa

A

Elastin gene

20
Q

Nonfamilial cutis laxa cases

A

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

Isolated acral dz associated with myeloma and RA

21
Q

De barsey

A

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

22
Q

Blepharochalasis

A

Lax eyelid skin, usually BL

Lack of elastic fibers and IgA deposits

23
Q

Ascher syndrome

A

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

24
Q

MC location for anetoderma

A

Shoulders, upper arms, thighs

25
Q

Anetoderma associations

A

Up to 1/2 have lupus, antiphospholipid ab, graves, scleroderma, low Cā€™, hypergamma, HIV

26
Q

Anetoderma of prematurity

A

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

27
Q

Striae

A

Decreased upper dermal collagen, lie parallel to epi

Increases elastin fibers

28
Q

Linear focal elastosis

A

Males
Increased elastic fibers
Fig 25.9

29
Q

OI

A

Lobstein syndrome

30
Q

Brack syndrome

A

OI + arthrogryposis