Icthyosis/ fish scale disease / collodion baby.
It is a genetics- autosomal recessive disorder, Affecting around 1 in 600,000 people due to deficiency of the enzyme keratinocyte transglutaminase- Keratinization disorders (hyperkeratosis).
Synonane of Collodion baby.
a. Fish
scale disease.
b. collodion baby.
Cause of Collodion baby.
Genetics-
autosomal recessive disorder.
Affecting
around 1 in 600,000 people.
Basic
defect- deficiency of the enzyme keratinocyte transglutaminase- Keratinization
disorders (hyperkeratosis).
Clinical feature of Collodion baby.
- Scanty
hair (may be absent).
- Ectropion
(lower eyelids turned outwards away from the eyeball).
- Flat
nose or ear.
- Eclabium-(O
shaped oral orifice)- the turning out of the lips due to the tightness of the
membrane.
- Extensive
scaling of the skin caused by hyperkeratosis.
- Very
hard, thick, scaly, parchment like skin.
- Dry
fissured skin.
- Limb
defect may be present.
Complication of Collodion baby.
- Fluid
loss- dehydration, hypovolemic hypernatremia.
- Hypothermia.
- Infection.
- Thick
skin causes mechanical problem-Suckling, breathing, aspiration pneumonia.
Treatment of Collodion baby.
- Nutritional
support- IV fluid, NG feeding.
- Regular
application of- emollients such as petrolatum gelly to keep the skin moist.
- Artificial
tears if there is severe ectropion.
- Infection control.
- Vitamin
e – 1 mg/kg/day.
- Consult
with a pediatric dermatologist
