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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