Paediatric dermatology

Question 1

What are the 2 categories of birthmarks in children

Answer 1

vascular

pigmented

Question 2

What are features of a salmon patch

Answer 2

central / midline location 
facial or nuchal 
due to persistent foetal circulation 
aka angels kisses
common, completely benign
macular 
erythematous 
very likely to disappear

Question 3

How can vascular birthmarks be divided

Answer 3

vascular malformation

haemangioma

Question 4

what are the groups of vascular malformation

Answer 4

Abnormality of any vessel
arterial - fast flow, dangerous, rare
venous - more common, slow flow 
Capillary 
lymphatic
mixture

Question 5

what are haemangiomas

Answer 5

vascular tumours confined to childhood

rapid growth then regression

Question 6

vascular malformations are more likely to regress than haemangiomas, true or false

Answer 6

false, haemangiomas have rapid growth but then regress in comparison to vascular malformations which do not resolve or may even progress

Question 7

vascular malformations are more likely to regress than haemangiomas, true or false

Answer 7

false, haemangiomas have rapid growth but then regress in comparison to vascular malformations which do not resolve or may even progress

Question 8

give an example of a capillary vascular malformation

Answer 8

port wine stain / naevus flammeus

Question 9

features of port wine stain (PWS)

Answer 9

dark red macular lesion 
do not extend beyond original borders 
lifelong persistence 
usually unilateral 
may thicken and darken
described in relation to the trigeminal nerve

Question 10

what syndromes may be associated with port wine stains?

Answer 10

Sturge weber syndrome especially in CNV1

risk of epilepsy development

Question 11

what syndromes may be associated with capillary malformation?

Answer 11

• Sturge weber syndrome PWS in CNV1 and ipsilateral vascular malformation in the brain –> seizures, glaucoma
• Klippel-Trenauney syndrome - capillary venous malformation and capillary-lymphatic-venous malformation, progressive limb overgrowth
  PWS on lower limb

Question 12

features of venous malformation/anomaly

Answer 12

bluish stain
if left untreated can progress severely
Mx with injection of sclerosants and coils

Question 13

another name for infantile haemangiomas

Answer 13

strawberry naevi

Question 14

what is the commonest vascular tumour in children

Answer 14

infantile haemangioma
benign soft tissue tumour
Can be superficial, deep to the skin or combined

Question 15

epidemiology of infantile haemangiomas

Answer 15

90% appear within the first month of life
80% single lesion
commonest on H+N
commoner in prems and females
most undergo total/partial resolution
if they occur across the midline - concerning

Question 16

complications of haemangiomas

Answer 16

pain 
infection 
bleeding 
ulceration 
visual obstruction 
airway/swallowing obstruction

Question 17

complications of peri ocular haemangiomas and their management

Answer 17

visual obstruction 
glocal compression 
retrobulbar expansion 
fast growing 
oral propranolol

Question 18

management of infantile haemangiomas

Answer 18

topical/IL steroids
topical propranolol
systemic: steroids, propranolol
propranolol needs

Question 19

what is the proper name for mongolian blue spots

Answer 19

slate grey naevus / congenital dermal melanocytosis

Question 20

features of slate grey naevi

Answer 20

bluish grey appearance
normal in black and asian skins
usually fade over a number of years

Question 21

differential of slate grey naevi

Answer 21

NAI

Question 22

what is a melanocytic naevus more commonly known as

Answer 22

mole

Question 23

features of melanocytic naevi

Answer 23

can become warty and hairy
larger than acquired naevi
grouped as small, medium and large

Question 24

what are bathing trunk naevi

Answer 24

giant congenital naevi

Question 25

what are Cafe Au Lait Macules (CALMS)

Answer 25

well defined light brown macules normal in 10-20% 2 CALMS under 2yo suggests neuroectodermal disease

Question 26

what is NF1 and what is its inheritance

Answer 26

commonest genodermatosis multisystem disorder AD inheritance with variable penetrance

Question 27

cutaneous features of NF1

Answer 27

neurofibromas Cafe au lait macules axillary (and groin) freckling = Crowe's sign

Question 28

what are Lisch nodules

Answer 28

hamartomas seen in the iris not harmful useful clinical sign for NF1

Question 29

list non-cutaneous features of NF1

Answer 29

``` ocular CNS skeletal tumours vascular fibrosing alveolitis ```

Question 30

describe an ash leaf macule and with which condition is it synonymous

Answer 30

oval shaped area of hypopigmentation tuberous sclerosis can use a wood's lamp to examine this

Question 31

inheritance of tuberous sclerosis

Answer 31

AD

Question 32

features of tuberous sclerosis

Answer 32

``` infantile seizures - cortical tubers ash leaf macule Shagreen patches facial angiofibromas periungal fibromas hamartomas angiomyolipomas CALMS bone cysts enamel pits in teeth ```

Question 33

What are shagreen patches

Answer 33

connective tissue naevi | orangey

Question 34

what are common inflammatory skin conditions in paediatrics

Answer 34

``` eczema psoriasis fungal infection acne vitiligo molluscum lichen sclerosis vulovaginitis morphea ```

Question 35

what is PWS described in relation to

Answer 35

CNV

Question 36

why is it important to distinguish between salmon patch and PWS

Answer 36

PWS in the CNV1 can have underlying epilepsy

Question 37

precursor lesion for haemangiomas

Answer 37

can appear slightly red and benign looking but it is underlying

Question 38

monitoring for propranolol

Answer 38

regular glucose and cardiac monitoring - HR and BP