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Flashcards in Dermal Connective Tissue Disorders Deck (29)
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1
Q

Treatment options for striae

A

Tretinoin 0.1%
10% Vit C with 20% glycolic acid
Pulsed dye laser reduce redness
308 nm excimer laser has helped with leukoderma

2
Q

What is atrophoderma of Perini and Pasini?

A

Usually first to second decade of life
F>M, reported in children <13y
Aetiology unknown - overlap with morphoea and percentage of European have positive Borrelia serology 40-50%
- oval to circular brown to blue/hypopigmented plaques NOT indurated or sclerotic
- appears on back / lumbosacral area, then chest/arms/abdomen.
- face, palms, scalp and soles are SPARED
- lesions have a ‘cliff drop’ sharp demarcation although sometimes this can be slanted
- depressed patches can give impression of inverted plateaus or when multiple have a Swiss cheese appearance
- occasionally dermal blood vessels can be seen

3
Q

Atrophoderma of Perini and Pasini - short natural history of 1-2 years

A

False - protracted 10-20y

4
Q

Follicular atrophoderma refers to dimple like depressions at follicular orifices

A

True

5
Q

list Genodermatoses related to follicular atrophoderma

A
  • Bazex-Dupre-Christol (follicular atrophoderma, milia, multiple BCC, X linked DOMINANT, localised hypohidrosis above neck, follicular atrophoderma - multiple ice pick marks on dorsal hands, lower back and elbows but RARELy on the face)
  • Conradi-Hunermann-Happle syndrome (X linked dominant chondrodysplasia punctata, females, lethal in males, ichthyosiform streaking in Blaschko lines, replaced by bands of follicular atrophoderma )
6
Q

Atrophoderma vermiculatum occurs in Bazex-Christol-Dupre

A

False - in Rombo and Loewy-Dietz syndrome

7
Q

Atrophoderma vermiculatum does not benefit from oral isotretinoin

A

False

8
Q

Piezogenic pedal papules assoc. with collagen disorders only

A

False - can see in normal population

9
Q

Papular elastorrhexis has so associated extracutaneous anomalies

A

True

10
Q

Papular elastorrhexis are more compressible and flaccid compared to anetoderma

A

False - more firm and non compressible

11
Q

Papular elastorrhexis are follicular papules 2-5 mm in diameter

A

False - non follicular , scattered on the trunk

12
Q

Jadassohn-Pellizzari type primary anetoderma has no preceding inflammatory lesions

A

False, this is Schweninger-Buzzi

13
Q

Primary anetoderma more common in males

A

False - females

14
Q

Mid dermal elastolysis related to UV exposure

A

True - possible relation to tanning

15
Q

Mid dermal elastolysis are discrete perifollicular papules

A

True

16
Q

2 types of mid dermal elastolysis

A
  1. TYPE I - with site of central hair follicle indented

2. TYPE II - occasionally erythematous patches, telangiectasias, and reticulated erythema can be present

17
Q

Cutis laxa can involves skin but not internal organs

A

False - involves internal organs

18
Q

List extracutaneous features of cutis laxa

A
Emphysema - pulmonary complications cause significant morbidity and mortality 
Umbilical and  inguinal hernias
GI and GU tract diverticula
Cardiac or arterial abnormalities
Skeletal or joint involvement
Delayed growth and development
19
Q

Cutis laxa genes?

A

Mutations in elastin (ELN) and FBN gene with recessive type 1

20
Q

Drugs that cause cutis laxa

A

Penicillamine, penicillin, SSRIs

21
Q

Marshall syndrome can occur mainly in teenagers and young adults post Sweets

A

False - infants and young children

22
Q

PXE = sometimes caused by ENPP1 mutations

A

True, mainly ABCC6 gene but also this one

23
Q

PXE ocular manifestations?

A

Peau d’orange
Angioid streaks
Optic drusen
Owl eyes - paired hyperpigmented spots, can be detected

24
Q

PXE CVS manifestations

A

Renovascular hypertension,
Myocardial infarction, angina pectorals
Calcification of vessels — risk thromboembolic events
Mitral valve prolapse

25
Q

PXE GI manifestations

A

Calcified blood vessels in gastric and intestinal mucosa —> incr propensity for GI haemorrhage
Young patient can present with GI bleeding particularly from the stomach

26
Q

GU /pregnancy manifestations of PXE

A
  • no incr risk of miscarriage

May be related to preterm labour however

27
Q

List perforating disorders

A
Perforating GA
Perforating PXE
Perforating folliculitis 
Elastosis perforans serpiginosa
Reactive perforating collagenosis
Perforating calcific elastosis
28
Q

Disorders with elastosis perforans serpiginosa

A

MADPORES

  • Marfan
  • ACROGERIA
  • Down syndrome
  • PXE and penicillamine
  • Osteogenesis imperfects
  • Rothmund-Thompson
  • Ehler’s Danlos syndrome
  • Scleroderma
29
Q

Fibromatosis superficial vs. deep

A

Superficial are Dupuytren (palmar), Ledderhouse (plantar) , Peyronie (penile), knuckle pads (haloderma, exists in Bart Pumphrey with hearing loss and leukonychia), pachydermodactyly (adolescent boys, relate to mechanical trauma. Soft tissue swelling of lat aspect of PIPJ of second to fourth fingers)