Regression of Urticaria Pigmentosa in Adult Patients With Systemic Mastocytosis

Brockow, Knut; Scott, Linda M.; Worobec, Alexandra S.; Kirshenbaum, Arnold S.; Akin, Cem; Huber, Mary; Metcalfe, Dean D.

doi:10.1001/archderm.138.6.785

Cited by 37 publications

(19 citation statements)

References 13 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…6,10,21,22 Mean tryptase levels have been discussed in prior studies to vary between 1 to 15 g/L in healthy control subjects, which reflect very well the results of our control group with an average tryptase level of 5.70 g/L. 21 A level greater than 20 g/L is strongly suggestive of a systemic mast cell disease, and has been included as minor criterion in the diagnosis of systemic mastocytosis (Table II).…”

Section: Discussionsupporting

confidence: 82%

Tryptase detection in bone-marrow blood: A new diagnostic tool in systemic mastocytosis

Proelss

Wenzel

et al. 2007

Journal of the American Academy of Dermatology

View full text Add to dashboard Cite

Section: Discussionsupporting

confidence: 82%

Tryptase detection in bone-marrow blood: A new diagnostic tool in systemic mastocytosis

Proelss

Wenzel

et al. 2007

Journal of the American Academy of Dermatology

View full text Add to dashboard Cite

“…In adults, the prognosis of indolent systemic mastocytosis is generally good, and in approximately 10% of the patients who have systemic mastocytosis for more than 10 years, urticaria pigmentosa may even regress [20]. In a study of 145 consecutive adult patients observed for a median of 147 months (range, 61-329 months), only 5 (3%) showed progression to a more aggressive form of the disease (aggressive systemic mastocytosis; systemic mastocytosis with an associated hematologic, non-mast cell lineage disease; or mast cell leukemia) [21••].…”

Section: Prognosismentioning

confidence: 99%

Update on Diagnosis and Treatment of Mastocytosis

Brockow

Ring

2011

Curr Allergy Asthma Rep

Self Cite

View full text Add to dashboard Cite

Mastocytosis is a disorder characterized by increased numbers of mast cells in tissues. Recent clinical observations highlight the association of mastocytosis with an increased risk of anaphylaxis and underline the diversity of this disease. At the molecular level, recent studies have attempted to unravel specific gene expression profiles for activating c-kit mutations in the etiology of mastocytosis. The diagnosis may be facilitated by surrogate markers and detection of aberrant immunophenotypic surface markers. New therapeutic strategies are in development based on intracellular signal pathways, or on application of topical treatments, as are novel forms of cytoreductive therapy, including tyrosine kinase inhibitors.

show abstract

“…Persistent CM of childhood and adulthood can lead to SM [22]. However, regression of UP in adult patients with ISM may or may not be accompanied by systemic disease improvement [23].…”

Section: Cutaneous Signs and Symptomsmentioning

confidence: 99%

Current Options in the Treatment of Mast Cell Mediator-Related Symptoms in Mastocytosis

Escribano¹,

Akin

Castells

et al. 2006

IADT

Self Cite

View full text Add to dashboard Cite

Patients with mastocytosis have symptoms related to the tissue response to the release of mediators from mast cells (MC), local mast cell burden or associated non-mast cell hematological disorders. MC contain an array of biologically active mediators in their granules, which are preformed and stored. MC are also able to produce newly generated membrane-derived lipid mediators and are a source of multifunctional cytokines. Mediator-related symptoms can include pruritus, flushing, syncope, gastric distress, nausea and vomiting, diarrhea, bone pain and neuropsychiatric disturbances; these symptoms are variably controlled by adequate medications. Management of patients within all categories of mastocytosis includes: a) a careful counseling of patients (parents in pediatric cases) and care providers, b) avoidance of factors triggering acute mediator release, c) treatment of acute and chronic MC-mediator symptoms and, if indicated, d) an attempt for cytoreduction and treatment of organ infiltration by mast cells.

show abstract

Regression of Urticaria Pigmentosa in Adult Patients With Systemic Mastocytosis

Abstract: To determine clinical correlates of urticaria pigmentosa (UP) regression in adult patients with systemic mastocytosis (SM).Design: Cohort study of the natural history of mastocytosis.

Cited by 37 publications

References 13 publications

Tryptase detection in bone-marrow blood: A new diagnostic tool in systemic mastocytosis

Tryptase detection in bone-marrow blood: A new diagnostic tool in systemic mastocytosis

Update on Diagnosis and Treatment of Mastocytosis

Current Options in the Treatment of Mast Cell Mediator-Related Symptoms in Mastocytosis

Contact Info

Product

Resources

About