A 6 year old child comes in with an itchy rash, particularly over the flexural areas such as their knees, wrists and ankles. They have asthma and is allergic to nuts. What is their diagnosis and management?
Atopic dermatitis = eczema
Management:
1) Emollients - soap substitutes
2) Topical corticosteroids - use lowest effective potency
3) Adjuncts i.e. topical calcineurin inhibitors e.g. tacrolimus/pimecrolimus
What are some triggers for atopic dermatitis?
Soaps
Heat
Stress
Dry skin
Infections
Allergens
Name 2 complications of eczema
Secondary infection from Staph aureus
Eczema herpeticum (HSV - emergency)
Sleep disturbance
Psychosocial impact
What is the difference between naevus simplex vs port wine stain?
Naevus simplex is a common, benign capillary malformation which fades spontaneously and requires no treatment
- Salmon patch
- Stork bite
Port Wine stain is darker, unilateral and does not fade
What are the key clinical features of port wine stain?
Congenital capillary malformation causing a flat, pink-purple vascular patch present at birth that does not regress.
What is an important association of port wine stain?
Sturge-Weber syndrome = congenital, non-inherited neurocutaneous disorder with vascular malformations affecting skin, brain and eyes
- Facial port wine stain in V1 distribution
What are the clinical features of Sturge Weber syndrome?
STURGE mnemonic:
Seizures
Tram-track calcifications (on neuroimaging)
Unilateral port-wine stain (commonly V1,V2 distribution of trigeminal nerve)
Retardation
Glaucoma
Epilepsy
What is the difference between port wine stain and infantile haemangioma?
Port wine stain is a congenital capillary malformation whereas infantile haemangioma is a vascular tumour
PWS has no growth phase or involution whereas infantile haemangioma does
Name 2 differentials for port wine stain
Infantile haemangioma
Naevus simplex
What is the management of port wine stain?
Medical:
- Ophthalmology review - glaucoma risk
- Neurology - if seizures/V1 involvement
- Community paediatrician - development
- Dermatology - pulsed dye laser (cosmetic improvement, best started early)
Psychological support
Would you treat port wine stain with propranolol?
NO
Propranolol is used for infantile haemangiomas NOT port wine stains
- PWS are capillary malformations and managed with laser therapy
What is an infantile haemangioma?
Benign, vascular tumour of infancy characterised by rapid postnatal proliferation followed by gradual spontaneous involution
Appearance:
- Bright red, raised
- Blanches with pressure
What is the natural progression of infantile haemangioma?
1) Proliferative phase: 0-6/9 months
2) Involution phase: begins after 1 year and continues over several years
- 50% resolved after 5 years; 90% resolved by 9 years
What are the red flags/complication of infantile haemangioma?
Location:
- Periorbital: visual impairment
- Airway (beard distribution): airway obstruction
- Large facial lesion: PHACE syndrome (rare condition linking infantile haemangioma with defects in brain, arteries, heart and eyes)
How many infantile haemangiomas put you at risk of heart failure and how?
5 or more
Hepatic haemangiomas can cause heart failure
What is the management of infantile haemangioma?
Conservative (majority): reassurance and regular review
Medical:
Propranolol
- Indicated if functional impairment, ulceration, cosmetic risk or airway/eye involvement
Topicol timolol (small superficial lesions)
Surgery/laser (selected cases)
What is important to monitor for in the context of starting propranolol?
Must have a baseline assessment first: HR, BP and glucose
Monitor for:
- Bradycardia
- Hypotension
- Hypoglycaemia
- Bronchospasm
Name 3 causes of cafe au lait spots
Neurofibromatosis type 1
McCune-Albright Syndrome
Legius syndrome
Noonan syndrome
How many cafe au lait spots are significant?
6 or more = NF1
Size:
- > 5 mm in pre-pubertal children
- > 15 mm in post-pubertal children
What is McCune Albright syndrome?
Cafe au lait spots + fibrous dysplasia (bone pain, fractures, deformity) + precocious puberty
What is vitiligo?
Acquired autoimmune condition causing well-demarcated depigmented macules due to destruction of melanocytes
Depigmented lesions (NOT hypopigmented)
What other conditions are vitiligo associated with?
T1DM
Autoimmune thyroid disease
Addison disease
Pernicious anaemia
What is the Koebner phenomenon?
Vitiligo at sites of trauma
What is albinism?
A genetic disorder of melanin production = generalised hypopigmentation of the skin, hair and eyes with visual impairment