Treatment and prognosis of cutaneous mastocytosis
- Mariana C Castells, MD, PhD
Mariana C Castells, MD, PhD
- Associate Professor of Medicine
- Harvard Medical School
- Cem Akin, MD, PhD
Cem Akin, MD, PhD
- Associate Professor of Medicine
- Harvard Medical School
- Director, Mastocytosis Center
- Brigham and Women's Hospital
Cutaneous mastocytosis describes a group of disorders characterized by the presence of excessive numbers of mast cells in the skin. Patients with cutaneous mastocytosis do not fulfill diagnostic criteria for systemic mastocytosis and show no evidence of organ involvement other than the skin.
Forms of cutaneous mastocytosis include three variants recognized by the World Health Organization (WHO) :
●Maculopapular cutaneous mastocytosis (MPCM) or urticaria pigmentosa (UP)
●Diffuse cutaneous mastocytosis
●Solitary cutaneous mastocytoma
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- GENERAL MEASURES
- Preparation for treating possible anaphylaxis
- Trigger avoidance
- Problematic medications
- Medications that are usually tolerated
- Premedication before anticipated trigger exposure
- Treatment of coexistent allergic disease
- - Hymenoptera venom hypersensitivity
- PHARMACOLOGIC THERAPIES
- Diffuse cutaneous disease and urticaria pigmentosa
- - Antihistamines
- - Cromoglycates
- - Leukotriene-modifying agents
- - Treatment of refractory symptoms
- - Therapies that are not recommended
- Cutaneous mastocytomas
- - Pharmacotherapy
- Infants and young children
- Older children and adults
- SUMMARY AND RECOMMENDATIONS