Paediatric dermatology Flashcards
Define what a birthmark is
This is an unusual, typically permanent, mark on someone’s body which is there from birth.
What is the importance of birthmarks?
- They may be a target for unkind comments
- & for medical staff they may suggest underlying problems and target further investigations
What are the 2 main groups of birthmarks ?
- Vascular
- Pigmented
What is a genodermatose?
It means the condition is an inherited dermatological disorder
What are the 2 main genodermatoses ?
- Neurofibromatosis
- Tuberous sclerosis
What are the 2 main groups of vascular birthmarks ?
- Capillary vascular malformations
- Haemangiomas
What are capillary vascular malformations ?
- These are congenital (present from birth) malformed dilated blood vessels in the skin.
- They appear as blotches of red or purple skin discolouration on any part of the body.
- They grow in proportion to the childs body
What are the 2 main capillary vascular malformations (vascular birthmarks) ?
- Salmon patches (naevus simplex)
- Port-wine stains (naevus flammeus).
What are the clinical features of salmon patches ?
- Small flat patches of pink or red skin with poorly defined borders.
- They are commonly found at the nape of the neck (stork bite, erythema nuchae), on the forehead between the eyebrows (angel’s kiss) or on the eyelids.
- They become more intense in colour and noticeable when the child is crying
How common are salmon patches ?
Very - occur in about 40% of all newborns
As time progresses what happens to salmon patches ?
Most lesions will spontaneously dissapear within the 1st year of life
What is the treatment of salmon patches ?
No treatment needed
What are the clinical features of port-wine stains ?
- A large flat patch of purple or dark red skin with well-defined borders.
- Tend to be unilateral
- In time it becomes bumpy and often more unsightly. The face is most commonly affected although they can occur anywhere on the body.
As time progresses what happens to port-wine stains ?
- Most remain unchanged or may even deepen in colour.
Hint- think of jorge to remember this condition
What is the treatment of port-wine stains ?
Cosmetic camouflage or laser therapy (multiple sessions are required).
Most capillary vascular naevi are not associated with other abnormalities. However, there are some rare syndromes that may present with this kind of birthmark, what are the 2 main ones to know about ?
- Sturge Weber Syndrome
- Klippel-Trenauney syndrome
What is sturge-weber syndrome ?
It is the association of capillary vascular malformation affecting the skin supplied by one branch of the trigeminal nerve of the face with defects in the underlying tissues.
This syndrome may result in:
- Seizures
- Intellectual impairment
- Hemi-paresis
- Eye abnormalities (glaucoma, optic atrophy) which can result in blindness
What is Klippel-Trenauney syndrome ?
- This refers to the association of a capillary vascular malformation with a venous and a lymphatic malformation.
- The lymphatic malformation can contain large fluid-filled cysts (macrocystic) or clusters of smaller ones (microcystic).
- The affected limb is wider and bulkier than the other side.
- This syndrome can become complicated by blood clots, seepage of lymph, infections (cellulitis), ulceration & bleeding
What is an infantile haemangioma ?
More commonly known as a strawberry naevus is benign (non-cancerous) tumour of soft tissue affecting cutaneous blood vessles.
What are the 3 main types of infantile haemangioma ?
- Superficial also called capillary haemangioma - the blood vessels in uppermost layers of the skin are dilated.
- Deep also called cavernous haemangiomas and are more deeply set in the dermis and subcutis. They appear as a bluish soft to firm swelling.
- Mixed type - when a superficial haemangioma overlies a bluish swelling.
What are the clinical features of infantile haemangiomas ?
- They appear as erythematous, raised and multilobed tumours.
- They are usually not present at birth but rapidly develop in the 1st month of life
- Typically they increase in size until around 6-9 months before regressing over the next few years (around 95% resolve before 10 years of age).
Where do infantile haemangiomas usually arise on the body ?
- The face, scalp and back.
- Rarely they may be present in the upper respiratory tract leading to potential airway obstruction
What complications can develop as a result of an infantile haemangioma ?
- Mechanical e.g. Obstructing visual fields or airway
- Bleeding
- Ulceration
- Thrombocytopaenia
If an infantile haemangioma develops over the spinal cord what condition should you be worried about and therefore what investigation should be done ?
- Potentially indicates spina bifida/ cord tethering lumbosacral area
- Do an ultrasound to rule out