Jan 18 2009

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Mast cell disease

 

 

What is mast cell disease?

Mast cells are normally found around our blood vessels.  An accumulation of excessive numbers of the mast cells in the skin is known as mastocytosis.  The mast cells contain chemicals including histamine, that may be released when the lesion is rubbed, in heat, or which certain medications.  In children, this disorder me appear as a single lesion - ” solitary mastocytoma” or more generalized forms termed ” anurticaria pigmentosa” or “mastocytosis”.  The lesions usually appear at birth or earlier childhood, may increase somewhat in size and number for several months to years, and eventually go away in most cases. Mastocytomas usually clear by school age.  The yellow-brown spots of urticaria pigmentosa may begin to clear at puberty.  Children with lesions, rarely have increased numbers of the mast cells at other sites then the skin.  Patients often development local redness and itchy hive-like areas over the lesions after gentle rubbing or stroking (Darier’s sign) due to the release of histamine in the mast cells.  Sometimes a small fluid-filled blister will appear always at the same spot.  When itching is a problem, an antihistamine, an agent that blocks histamine, and such as Benadryl, may be given.  If problems such as flushing, dizziness, irritability, diarrhea or extensive swelling occur frequently, long-term use of antihistamine agents may be indicated.

 

Irritants for children with mast cell disease

  • Physical stimuli: exercise; skin friction; hot baths; cold exposure (especially swimming); ingestion of hot beverages, spicy foods or ethanol
  • Drugs: aspirin, alcohol, morphine, codeine, Polymyxin B, thiamine, quinine, D-tubocurarine, radiographic dyes scopolamine, procaine, opiates, nonsteroidal anti-inflammatory agents, gallamine, decamethonium.
  • Others: IV high molecular weight polymers (dextran), emotional stress, bacterial toxins, snake venoms, polypeptides released by ascaris, jellyfish, crayfish and lobsters.

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