Bullous mastocytosis

    Article Contributors: 
    Amr Ismail MD , Ph.D...

     

    Mastocytosis is characterised by disorderly mast cell infiltration of the skin and other tissues.
    WHO classification showed cutaneous,indolent and aggressive systemic and malignant diseases.
    Bullous mastocytosis(BM):
    Rare variant of cutaneous mastocytosis that usually affects infants during 1st year of life
    with male preference.BM of chilhood is severe form with risk of shock and sudden death.
    Characterised clinically by skin lesions A)diffuse blisters of the body B) Severe itching.
    and GIT symptoms: vomiting ,abdominal pain and diarrhea.
    Histologically: subepidermal blister with mast cells.
    References:
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    Pediatric mastocytosis.
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    2. Hartman K, Henz BM.
    Mastocytosis: Recent advances in defining the disease.
    Br J Dermatol 2001;144:682-95.

    3. Valent P.
    Diagnostic evaluation and classification of mastocytosis.
    Immunol Allergy Clin North Am. Aug 2006;26(3):515-34.

    4. Patnaik MM, Rindos M, Kouides PA, Tefferi A, Pardanani A.
    Systemic mastocytosis: a concise clinical and laboratory review.
    Arch Pathol Lab Med. May 2007;131(5):784-91.