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Dermatology > Paediatric Dermatology > Flashcards

Paediatric Dermatology Flashcards

1
Q

What are salmon patches?

A

Vascular birthmarks believed to be due to persistent foetal circulation rather than a malformation

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2
Q

How common are salmon patches?

A

Common - up to 50% of new borns

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3
Q

Where do salmon patches typically present?

A

Facial - eyelids, forehead, nose

Nuchal

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4
Q

Are facial or nuchal salmon patches more persistent?

A

Nuchal (salmon patches tend to resolve but 10% nuchal salmon patches are present in 10% of adults)

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5
Q

What are facial salmon patches also known as?

A

Angel’s kisses

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6
Q

What are nuchal salmon patches also known as?

A

Stork marks

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7
Q

What are the 2 types of vascular birthmarks?

A

Vascular malformation

Haemangioma

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8
Q

Does vascular malformation resolve?

A

No - may progress

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9
Q

Do haemangiomas resolve?

A

Yes - most will regress and disappear to some extent

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10
Q

What is an haemangioma?

A

Vascular tumours confined to children

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11
Q

What is the most common vascular malformation?

A

Port-wine stains (Naevus flammeus)

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12
Q

Do port wine stains regress?

A

No - they persist for life

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13
Q

On the face PWS are described with references to which nerve?

A

Trigeminal (CN V)

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14
Q

What is Sturge Weber Syndrome?

A

A neurological disorder consisting of PWS of the ophthalmic region of the trigeminal nerve and ipsilateral vascular malformation of the brain

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15
Q

What are the complications Sturge Weber Syndrome?

A

Seizures - common (20% of V1 PWS)
Intellectual impairment
Hemi-paresis
Glaucoma - up to 45% (especially if upper and lower eyelids involved)

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16
Q

What are the triad of classical features in Klippel-Trenauney Syndrome?

A
  1. Port wine stain (usually on the limb)
  2. CMV (capillary venous malformation) and CLVM (capillary lymphatic venous malformation)
  3. Soft tissue hypertrophy of affected limb
17
Q

What is the commonest vascular tumour which occurs in childhood?

A

Infantile haemangioma (Strawberry naevus)

18
Q

When do most infantile haemangiomas appear?

A

90% appear within the first month

19
Q

Where do infantile haemangiomas most commonly present?

A

Head and neck

20
Q

What are the complications of infantile haemangiomas?

A

Bleeding
Ulceration
Infection
Pain

21
Q

What is the treatment of peri-ocular lesions?

A 
Topical propranolol (may also require intralesional steroid)
Prednisolone may be required if good improvement is not made
22
Q

If spina bifida is suspected was investigation should be performed?

A

Ultrasound

23
Q

If an infant is prescribed propranolol what kind of screening and monitoring is required?

A

Glucose and cardiac screening with regular monitoring of pulse and blood pressure

24
Q

A melanocytes naves is most commonly called what?

A

A mole

25
Q

Congenital melanocytic naevi have a 4 fold increased risk of developing what?

A

Melanoma (higher for large lesions)

26
Q

What are the sizes of a small, medium and large congenital melanocytic naevi?

A

Small - up to 5cm
Medium - between 5-20cm
Large - over 20cm

27
Q

What is the commonest genodermatosis?

A

Neurofibromatosis (NF Type 1)

28
Q

Is NF type 1 autosomal dominant of recessive?

A

Dominant: variable penetrance. Ch 17

29
Q

What is the pathophysiology or NF type 1?

A

Defect in amino acid which inactivates oncogenes

30
Q

What is present in 90% of NF type 1 patients?

A

Café au lait macules (CALMS)

31
Q

Along with CALMS, what is the other cutaneous lesion associated with NF type 1?

A

Neurofibroma

32
Q

What is the minimal diagnostic criteria for NF1?

A

Two or more:

  • 6 or more CALMS > 5 mm prepuberty or > 1.5cm adults
  • 2 or more Neurofibromas or 1 plexiform neuroma
  • Axillary (Crowe’s sign) or inguinal freckling
  • Optic glioma
  • 2 or more Lisch nodules
  • A distinctive bony lesion e.g. sphenoid dysplasia
  • A first-degree relative with NF1
33
Q

What is an ash leaf macule?

A

Oval shaped area of hypopigmentation, synonymous with Tuberous sclerosis

34
Q

What is the second most common genodermatosis after NF 1?

A

Tuberous sclerosis

35
Q

What neurological symptoms may be present in tuberous sclerosis?

A

Seizures
Intellectual disability
Developmental delay
Behavioral problems

36
Q

What is the earliest sign of tuberous sclerosis?

A

Ash leaf macule

37
Q

Is tuberous sclerosis autosomal dominant or recessive?

A

Dominat: 9q34 or 16p13.3

38
Q

What is the pathophysiology of tuberous sclerosis?

A

Abnormalities in cell cycle regulation genes

allowing hamartoma development

39
Q

What are the potential features of tuberous sclerosis?

A
  1. Depigmented macules (90%)
  2. CALMS in 25%
  3. Seizures: Cortical tuber, calcification of falx cerebra
  4. Facial angiofibromas (adenoma sebaceum)
  5. Periungual fibromas
  6. Shagreen patches (seen in 50%)
  7. Fibrous forehead plaques
  8. Enamel pitting
  9. Hamartomas / angiomyolipomas: Heart, lung kidneys
  10. Bone cysts

Dermatology (16 decks)

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