Systemic mastocytosis
ICD-10 D47.0 · ICD-11 2A21.0

Treatment of Indolent or Smoldering Systemic Mastocytosis with Pruritus and Skin Flushing

This protocol addresses patients with indolent or smoldering systemic mastocytosis (ISM/SSM) who experience pruritus and/or flushing of the skin — symptoms driven by mast cell mediator release. Skin-directed symptom control is a central management priority in this setting.

In indolent and smoldering forms of systemic mastocytosis, patients often present without significant organ damage but carry a meaningful symptom burden. Cutaneous manifestations such as pruritus and flushing are among the most common complaints. Treatment goals for ISM patients are primarily directed toward anaphylaxis prevention, symptom control, and osteoporosis management.

The protocol involves a leukotriene antagonist-based intervention targeting refractory skin symptoms. This represents a second-line approach when initial symptom management proves insufficient.

Specific agent selection, sequencing, and clinical decision criteria are available in the full protocol below.

References

DOI: 10.1002/ajh.26962

  • Treatment goals for ISM patients are primarily directed toward anaphylaxis prevention/symptom control/osteoporosis treatment.
  • Montelukast 10 mg/d
  • Zafirlukast 20 mg BID

View source ↗