title: Assessment of the extent of cutaneous involvement in children and adults with mastocytosis: relationship to symptomatology, tryptase levels, and bone marrow pathology.
abstract: BACKGROUND
  Cutaneous involvement occurs in most patients with systemic mastocytosis.
  
  
  OBJECTIVE
  We sought to determine whether the extent of cutaneous involvement is predictive of systemic disease.
  
  
  METHODS
  In a prospective survey of 48 adults and 19 children, the extent and density of cutaneous lesions were compared with patient history, symptoms, internal organ involvement, serum total mast cell tryptase level, and bone marrow pathology.
  
  
  RESULTS
  Cutaneous lesions in children were of a greater mean and maximum diameter, but similar in extent and density compared with lesions in adults. In adults with skin lesions, the extent of lesions correlated to disease duration. Adults with extensive cutaneous disease experienced more pruritus and flushing. Fatigue, splenomegaly, and hepatomegaly were more frequent in adults without cutaneous involvement; and in those with a greater density of lesions and disease duration. Increased tryptase levels were found in children and adults with systemic disease and correlated to skin lesion density and bone marrow pathology.
  
  
  CONCLUSIONS
  An examination of the extent and density of cutaneous lesions in adults helps identify those with more extensive extracutaneous disease and, thus, requiring a more thorough evaluation.
authors.affiliation: Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892-1881, USA.
authors.forename: Knut
authors.initials: K
authors.lastname: Brockow
authors.forename: Cem
authors.initials: C
authors.lastname: Akin
authors.forename: Mary
authors.initials: M
authors.lastname: Huber
authors.forename: Dean D
authors.initials: DD
authors.lastname: Metcalfe
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001853:Bone Marrow / Q000473:pathology; D002648:Child; D005260:Female; D006529:Hepatomegaly / Q000150:complications; D006801:Humans; D008297:Male; D034721:Mastocytosis, Systemic / Q000150:complications / Q000175:diagnosis / Q000201:enzymology / Q000473:pathology*; D008875:Middle Aged; D011446:Prospective Studies; D012697:Serine Endopeptidases / Q000097:blood; D012867:Skin / Q000473:pathology; D012871:Skin Diseases / Q000473:pathology*; D013163:Splenomegaly / Q000150:complications; D053802:Tryptases
chemical_list: D012697:Serine Endopeptidases; D053802:Tryptases