eMedicine Specialties > Dermatology > Benign Neoplasms

Mastocytosis: Follow-up

Author: Daniel J Hogan, MD, Clinical Professor of Internal Medicine (Dermatology), NOVA Southeastern University; Investigator, Hill Top Research, Florida Research Center
Coauthor(s): Christin Martino, Louisiana State University School of Medicine-Shreveport
Contributor Information and Disclosures

Updated: Sep 11, 2009

Follow-up

Further Inpatient Care

Emergency resuscitation or hospitalization may be required for severe syncope or hypotensive shock resulting from the sudden severe degranulation of many mast cells.

Inpatient & Outpatient Medications

Advise mastocytosis patients with systemic disease or extensive symptoms to wear a medical alert bracelet and carry injectable epinephrine in case of an acute event causing mast cell degranulation and subsequent shock. Adults have been reported with severe anaphylaxis after insect stings especially hymenoptera stings.18

Complications

  • Possible transformation into a hematologic malignancy
  • Death secondary to mast cell degranulation
  • Mast cell leukemia

Prognosis

The prognosis depends on the age of onset. Most patients with urticaria pigmentosa (UP) exhibit onset before age 2 years, which is associated with an excellent prognosis, often with resolution by puberty. The number of lesions diminishes by approximately 10% per year.

Cutaneous mastocytosis onset after age 10 years portends a poorer prognosis, because late-onset disease tends to be persistent, is associated more often with systemic disease, and carries a higher risk of malignant transformation.

In patients with systemic mastocytosis, regression of urticaria pigmentosa is associated with a decreased frequency and severity of other symptoms. Bone marrow findings do not change with regression of urticaria pigmentosa.

Patient Education

Instruct cutaneous mastocytosis patients about avoiding physical stimuli and substances that trigger the condition. Additionally, educate patients and/or parents about the signs and treatment of anaphylaxis, especially in patients with systemic disease or severe symptoms.

Miscellaneous

Medicolegal Pitfalls

Cutaneous mastocytosis lesions in children have been mistaken for skin signs of child abuse.

 
Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author, Virginia Pylant Lewis, MD, to the development and writing of this article.



More on Mastocytosis

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References
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Further Reading

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Keywords

mastocytosis, mastocytoma, telangiectasia macularis eruptiva perstans, TMEP, urticaria pigmentosa, mast cell proliferation

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Contributor Information and Disclosures

Author

Daniel J Hogan, MD, Clinical Professor of Internal Medicine (Dermatology), NOVA Southeastern University; Investigator, Hill Top Research, Florida Research Center
Daniel J Hogan, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Contact Dermatitis Society, and Canadian Dermatology Association
Disclosure: Nothing to disclose.

Coauthor(s)

Christin Martino, Louisiana State University School of Medicine-Shreveport
Christin Martino is a member of the following medical societies: American Medical Association and Student National Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Timothy McCalmont, MD, Director, UCSF Dermatopathology Service, Professor of Clinical Pathology and Dermatology, Departments of Pathology and Dermatology, University of California at San Francisco
Timothy McCalmont, MD is a member of the following medical societies: Alpha Omega Alpha, American Medical Association, American Society of Dermatopathology, California Medical Association, College of American Pathologists, and United States and Canadian Academy of Pathology
Disclosure: Apsara Consulting fee Independent contractor

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA
Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Rosalie Elenitsas, MD, Herman Beerman Associate Professor of Dermatology, University of Pennsylvania School of Medicine; Director, Penn Cutaneous Pathology Services, Department of Dermatology, University of Pennsylvania Health System
Rosalie Elenitsas, MD is a member of the following medical societies: American Academy of Dermatology and American Society of Dermatopathology
Disclosure: Nothing to disclose.

CME Editor

Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania
Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology
Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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