Recurrent syncope and anaphylaxis as presentation of systemic mastocytosis in a pediatric patient: case report and literature review

J Am Acad Dermatol. 2006 May;54(5 Suppl):S210-3. doi: 10.1016/j.jaad.2005.06.012.


Mastocytosis refers to a rare collection of disorders, both cutaneous and systemic, that are characterized by increased numbers of mast cells. Depending on the extent of the disease, these disorders may present with symptoms resulting from mast cell degranulation including flushing, diarrhea, vomiting, cramping, syncope, or anaphylaxis. In pediatric patients, cutaneous involvement is most prevalent in the form of urticaria pigmentosa, which is typically asymptomatic or minimally so with resolution by adolescence. In this case report and review of literature, we review a case of a 3-year-old child with uritcaria pigmentosa displaying recurrent syncope and anaphylaxis as the first presentation of systemic mastocytosis. We found data to be limited on this topic, and concluded that pediatric patients with prior diagnoses of cutaneous mastocytosis could benefit from either more aggressive screening for systemic disease or prophylactic treatment with antihistamines and rescue subcutaneous epinephrine.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Anaphylaxis / etiology*
  • Cetirizine / therapeutic use
  • Child, Preschool
  • Histamine H1 Antagonists, Non-Sedating / therapeutic use
  • Histamine H2 Antagonists / therapeutic use*
  • Humans
  • Male
  • Mastocytosis, Systemic / complications*
  • Mastocytosis, Systemic / diagnosis
  • Mastocytosis, Systemic / drug therapy
  • Ranitidine / therapeutic use
  • Recurrence
  • Syncope / etiology*
  • Urticaria Pigmentosa / etiology*


  • Histamine H1 Antagonists, Non-Sedating
  • Histamine H2 Antagonists
  • Ranitidine
  • Cetirizine