title: Serum tryptase and SCORMA (SCORing MAstocytosis) Index as  disease severity parameters in childhood and adult cutaneous  mastocytosis.
abstract: BACKGROUND
  Skin lesions are the predominant clinical feature of the commonest form of mastocytosis. Mastocytosis is classified according to World Health Organization criteria. Determination of the levels of mast-cell mediators or their metabolites reflects the mast-cell burden. The extent of cutaneous mastocytosis can be assessed clinically using a scoring system (SCORing MAstocytosis; SCORMA Index) that we have developed.
  
  
  OBJECTIVE
  Serum tryptase levels were compared with the SCORMA Index in a large group of paediatric and adult patients to investigate whether there was any correlation between the two.
  
  
  METHODS
  The SCORMA Index in 64 patients (31 children and 33 adults) was compared with serum tryptase levels. The results of the first visit at which SCORMA and tryptase were evaluated were analysed.
  
  
  RESULTS
  There was a positive correlation between the SCORMA Index and serum tryptase levels, indicating the value of the SCORMA Index in the assessment of mastocytosis with skin involvement.
  
  
  CONCLUSIONS
  The results of this study showed that the SCORMA Index is a useful tool for evaluating the severity of cutaneous mastocytosis. The correlation between the SCORMA Index and serum tryptase levels underlines the benefit of the SCORMA Index as a clinical tool. Repeated SCORMA Index measurements can provide a rapid impression of changes in the clinical state of mastocytosis. This is particularly relevant in children, because taking blood samples from this group is much more difficult. The well-established methods for evaluation of disease severity may be expanded by the rapid SCORMA Index method.
authors.affiliation: Department of Dermatology, Medisch Centrum Alkmaar, JD Alkmaar, The Netherlands.
authors.forename: R
authors.initials: R
authors.lastname: Heide
authors.forename: K
authors.initials: K
authors.lastname: van Doorn
authors.forename: P G
authors.initials: PG
authors.lastname: Mulder
authors.forename: A W
authors.initials: AW
authors.lastname: van Toorenenbergen
authors.forename: A
authors.initials: A
authors.lastname: Beishuizen
authors.forename: H
authors.initials: H
authors.lastname: de Groot
authors.forename: B
authors.initials: B
authors.lastname: Tank
authors.forename: A P
authors.initials: AP
authors.lastname: Oranje
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D015415:Biomarkers / Q000097:blood; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008407:Mast Cells / Q000201:enzymology / Q000473:pathology; D034701:Mastocytosis, Cutaneous / Q000201:enzymology* / Q000235:genetics / Q000473:pathology*; D008875:Middle Aged; D011379:Prognosis; D012720:Severity of Illness Index; D053802:Tryptases / Q000097:blood*; D014582:Urticaria Pigmentosa / Q000201:enzymology / Q000235:genetics / Q000473:pathology*; D055815:Young Adult
chemical_list: D015415:Biomarkers; D053802:Tryptases