Bullous mastocytosis

     

    Introduction:

     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.

    Clinical Presentation:

    Characterised by

    (I)skin lesions A)diffuse blisters of the body B) Severe itching.

    and (II) GIT symptoms: vomiting ,abdominal pain and diarrhea. 

    Histological features:

    Subepidermal blister with mast cells.

    References:

     1. Kettelhut BV, Metcalfe DD. 

    Pediatric mastocytosis. 

    Ann Allergy 1994;73:197-207. 

    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.