Mastocytosis Treatment & Management

  • Author: Daniel J Hogan, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jun 4, 2010
 

Medical Care

Therapy is conservative and aimed at symptom relief because the prognosis for most patients with cutaneous mastocytosis (CM) is excellent. None of the currently available therapeutic measures induces permanent involution of cutaneous or visceral lesions. Advise patients to avoid agents that precipitate mediator release, such as aspirin, nonsteroidal anti-inflammatory drugs, codeine, morphine, alcohol, thiamine, quinine, opiates, gallamine, decamethonium, procaine, radiographic dyes, dextran, polymyxin B, scopolamine, and D-tubocurarine.

H1 and H2 antihistamines decrease pruritus, flushing, and GI symptoms. Oral disodium cromoglycate may ameliorate cutaneous symptoms, such as pruritus, whealing, and flushing, as well as systemic symptoms, such as diarrhea, abdominal pain, bone pain, and disorders of cognitive function.

Cautious administration of aspirin to inhibit prostaglandin synthesis and maintain mast cell degranulation may be beneficial for patients with disease resistant to H1 and H2 antagonist therapy alone. The patient, premedicated with H1 and H2 antihistamines, may be started on small doses of aspirin, slowly titrated to reach a plasma level of 20-30 mg/100 mL. Initiate this treatment regimen in a controlled environment, because aspirin can induce mast cell mediator release and subsequent cardiovascular collapse.

Cutaneous lesions that involve a limited body area may be treated with a potent class 1 topical corticosteroid, with occlusion if required. Intralesional injections of small amounts of dilute corticosteroids may resolve skin lesions temporarily or indefinitely. The risk of skin atrophy and adrenocortical suppression resulting from the treatment is minimized by treating limited body areas during a single treatment session. Systemic corticosteroids are useful only in specific situations, such as ascites, malabsorption, and severe skin disease. Case reports have described complete remission of bullous mastocytosis with oral corticosteroids,[25] but systemic therapy is often disappointing because the primary mode of action of corticosteroids is redistribution rather than death of mast cells.

Oral psoralen plus UV-A (PUVA) therapy results in general and cosmetic benefits in the treatment of cutaneous mastocytosis,[26] particularly telangiectasia macularis eruptiva perstans (TMEP); however, risks are involved, such as skin cancer if more than 200 treatments are required. The manifestations of the disease usually recur several months after discontinuation of PUVA, but recurrences respond as well as the original lesions. PUVA rarely is required in children. Currently, PUVA is reserved for severe, unresponsive cases in adults. In a 2003 study, medium-dose UV-A1 therapy was shown to be as effective as high-dose UV-A1 in reducing symptoms and the number of mast cells in affected skin.[27]

Medical alert bracelets should be made available, and an epinephrine self-injector demonstration should be performed and self-injector prescribed for patients with systemic mastocytosis or patients with episodes of vascular collapse. Patients with recurrent episodes of vascular collapse may be prophylactically placed on H1 and H2 antihistamines to lessen the severity of attacks. Episodes of vascular collapse may be spontaneous but have also occurred after stings from insects or after administration of iodinated contrast media for imaging studies. Premedication with antihistamines and corticosteroids is recommended before such procedures in patients at risk.

A clinical guideline summary from the American Academy of Allergy, Asthma and Immunology, The diagnosis and management of anaphylaxis: an updated practice parameter, may be helpful.[28]

General anesthesia may be problematic in patients with systemic mastocytosis and perioperative modalities of these patients need to be considered.[29] The treatment algorithm for systemic mastocytosis is complex, and the condition is primarily managed by a hematologist.[29, 30]

Future novel therapy for cutaneous mastocytosis includes immune modulators. One successful case report from 2008 described treatment of progressive c-kit mutation cutaneous mastocytosis with imatinib, halting disease symptoms and progression and improving the length of disease course.[31] Imatinib is approved by the Food and Drug Administration for use in patients with aggressive systemic mastocytosis with organ dysfunction due to progressive infiltration of various organs by mast cells without D816V c-kit mutation or unknown c-kit mutation status.[11]

A case report from 2008 described significant improved in cutaneous mastocytosis symptoms, especially in wheal formation, with anti-immunoglobulin E therapy in a patient with cutaneous mastocytosis and Ménière disease.[32]

Treatment of cutaneous mast cell lesions with the 585-nm flashlamp-pumped dye laser and the 532-nm Nd:YAG laser has been reported to provide cosmetic improvement.[33, 34]

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Consultations

Consultation with a hematologist may be necessary for a bone marrow biopsy and staging. The WHO classification of systemic mastocytosis mandates a number of staging investigations to define the exact subtype of disease. Identification of "B" findings alone, such as the following, is indicative of a high systemic mastocytosis burden.

  • Greater than 30% bone marrow mastocytosis burden
  • Serum tryptase level greater than 200 ng/mL

The additional presence of "C" findings, such as the following, is diagnostic for the presence of aggressive disease.[2]

  • Absolute neutrophil count less than 1000/µL
  • Hemoglobin value less than 10 g/µL
  • Platelet count less than 100 000/µL
  • Hepatomegaly with ascites and impaired liver function
  • Palpable splenomegaly with hypersplenism
  • Malabsorption with hypoalbuminemia and weight loss
  • Large-sized osteolysis or severe osteoporosis causing pathologic fractures or life-threatening organopathy in other organ systems definitively caused by an infiltration of the tissue by neoplastic mast cells
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Diet

Traditionally, physicians have advised cutaneous mastocytosis patients to avoid substances that induce mast cell mediator release, such as salicylates, crawfish, lobster, alcohol, spicy foods, hot beverages, and cheese. While evidence indicates that alcohol can cause adverse reactions, the role of the other foods mentioned above is hypothetical at this point and merits further investigation.[35]

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Activity

Advise cutaneous mastocytosis patients to avoid certain physical stimuli, including emotional stress, temperature extremes, physical exertion, bacterial toxins, envenomation by insects to which the patient is allergic, and rubbing, scratching, or traumatizing the lesions of cutaneous mastocytosis.

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Contributor Information and Disclosures
Author

Daniel J Hogan, MD  Clinical Professor of Internal Medicine (Dermatology), Nova Southeastern University College of Osteopathic Medicine; 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 M Mastrodomenico  Louisiana State University School of Medicine-Shreveport

Christin M Mastrodomenico is a member of the following medical societies: American Medical Association, Louisiana State Medical Society, and Student National Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Timothy McCalmont, MD  Director, UCSF Dermatopathology Service, Professor of Clinical Pathology and Dermatology, Departments of Pathology and Dermatology, University of California at San Francisco; Editor-in-Chief, Journal of Cutaneous Pathology

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

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster 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.

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: Lippincott Williams Wilkins Royalty Textbook editor; DLA Piper Consulting fee Consulting

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 Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

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.

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Urticaria pigmentosa lesions on the face of a child. Courtesy of Lee H. Grafton, MD.
Urticaria pigmentosa lesions on the back of a child. Courtesy of Lee H. Grafton, MD.
Lesion on the scalp of an infant.
Lesion on the arm. Courtesy of Lee H. Grafton, MD.
Blistering lesion.
Hematoxylin and eosin stain revealing mast cells in the papillary dermis.
Giemsa stain revealing mast cells.
 
 
 
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