Transcript Pediatric Mast Cell Disease - Mrs. Greil's Science Classroom

Pediatric Mast
Cell
Disease
By: Mrs. Greil
Sample Presentation
Pediatric Mastocytosis
A group of rare disorders in children
caused by the presence of too
many mast cells
There are three forms of pediatric
mastocytosis that are limited to
skin
Pediatric Mastocytosis
– Urticaria Pigmentosa – spots
– Mastocytoma – one bigger spot
(sometimes confused with a birth
mark)
– Diffuse cutaneous – Diffuse
involvement of the skin
Thomas (DCM) – you can see a healing
blister under his chin
Jake (UP) – characterized with
spots
Diagnosis of Diffuse Cutaneous
Mastocytosis (DCM)
• Children with DCM are diagnosed a couple of
ways.
– Skin biopsy
– Darrier sign
– Presence of skin lesions
Because mastocytosis is very rare often times children
are mis-diagnosed numerous times.
Symptoms of Diffuse Cutaneous
Mastocytosis (DCM)
• Symptoms (vary with the individual):
– Urticaria
– Darrier Sign
– Skin Lesions
– Blisters
– Itching
– Flushing
– Anaphalaxis
Causes of Mastocytosis
• DCM is caused by an excess of mast cells
found in the skin. These mast cells release
histamine (which can cause many symptoms
of mastocytosis).
• The cause for the infiltration of mast cells is
still unknown.
Treatment
• H1 and H2 blockers are used to treat the
symptoms of mastocytosis (antihistamines).
• Topical lotions or steroids
• Oral cromolyn
• Amongst many others
Treatment is different for every child depending on
the severity of the disease. As of today there is no
cure
Treatment Continued
• Treatment also involves a lifestyle change
– Staying away from mast cell degranulators such as
•
•
•
•
•
Aspirin
Alcohol
Certain fruits
Certain dyes
Etc….
– Temperature changes and friction from clothing
can lead to degranulation
– Carry an epipen!
Prognosis
• For children who are diagnosed with the
cutaneous form of mastocytosis before the
age of 1 the prognosis is good.
• 50% of the cases will spontaneously regress by
puberty.
• However some may develop into the systemic
form (which is a whole other presentation!)
Watch For More Information
Pediatric Mastocytosis
Sources
• List your sources/websites here
• Yes some of my information is minimal, but
thats because this is a sample!!
• Email me if you have any questions