eMedicine Specialties > Dermatology > Benign Neoplasms

Mastocytosis: Treatment & Medication

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

Treatment

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,21 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,22 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.23

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.24

General anesthesia may be problematic in patients with systemic mastocytosis and perioperative modalities of these patients need to be considered.25 The treatment algorithm for systemic mastocytosis is complex, and the condition is primarily managed by a hematologist.25,26

Future novel therapy for cutaneous mastocytosis includes immune modulators. One successful case report in 2008 described treatment of progressive c-kit mutation cutaneous mastocytosis with imatinib halting disease symptoms and progression and improving the length of disease course.27 Another case report in 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.28

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.29,30

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

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.31

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.

Medication

Tailor therapy according to the patient's specific needs for symptomatic relief. Patients may be started on oral H1 antihistamine and/or oral disodium cromoglycate with or without the use of a potent topical steroid for selected cutaneous lesions. In patients with a limited number of lesions, intralesional corticosteroid injections are an additional therapeutic option. Oral histamine H2 antagonists may be administered in addition to an oral H1 antihistamine.

Antihistamines

Relieve pruritus and flushing.


Hydroxyzine (Atarax, Atozine, Vistaril)

Antagonizes H1 receptors in periphery. May suppress histamine activity in subcortical region of CNS.

Adult

25-100 mg PO hs or divided qid

Pediatric

<6 years: 0.7 mg/kg/dose PO tid
>6 years: 50-100 mg/d PO in divided doses

CNS depression may increase with alcohol or other CNS depressants

Documented hypersensitivity; early pregnancy; breastfeeding

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Associated with clinical exacerbations of porphyria (may not be safe for patients with porphyria); ECG abnormalities (alterations in T waves) may occur; may cause drowsiness (caution patients about performing tasks requiring alertness)

Mast cell stabilizers

Cromolyn is a mast cell stabilizer that improves diarrhea, flushing, headaches, vomiting, urticaria, abdominal pain, nausea, and itching in some patients.


Cromolyn sodium (Gastrocrom)

Inhibits degranulation of sensitized mast cells following exposure to specific antigens. Available as an oral concentrate solution in 5-mL ampules to mix with water and is administered at least 30 min ac. Each 5-mL ampule contains 100 mg of cromolyn sodium, USP, in purified water. Each foil pouch contains 8 ampules.

Adult

200 mg PO qid 0.5 h ac and hs

Pediatric

Premature to term infants: Not recommended
<2 years: 20 mg/kg/d PO divided qid (attempt only in severe disease)
2-12 years: 100 mg PO qid 0.5 h ac and hs

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Do not use in severe renal or hepatic impairment; symptoms may recur when withdrawing drug

Histamine H2 antagonists

Addition of H2 antagonist to H1 antagonist helps control pruritus and whealing. In addition, GI symptoms associated with hyperchlorhydria, such as peptic ulcer disease and gastritis, respond well to this medication.


Cimetidine (Tagamet)

H2 antagonist that when combined with an H1 type may be useful in treating itching and flushing in urticaria and contact dermatitis that do not respond to H1 antagonists alone. Use in addition to H1 antihistamines.

Adult

300 mg PO qid with meals and hs

Pediatric

20-40 mg/kg/d PO in divided doses

Can increase blood levels of theophylline, warfarin, TCAs, triamterene, phenytoin, quinidine, propranolol, metronidazole, procainamide, and lidocaine

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Symptomatic response does not preclude malignancy; reversible CNS effects are possible; elderly patients may experience confusional states; may cause impotence and gynecomastia in young males; may increase levels of many drugs; adjust dose or discontinue if changes in renal function occur

Vasopressors

For patients with systemic disease or extensive symptoms.


Epinephrine (EpiPen)

DOC for treating anaphylactoid reactions. Has alpha-agonist effects that include increased peripheral vascular resistance, reversed peripheral vasodilatation, systemic hypotension, and vascular permeability. Beta-agonist effects include bronchodilatation, chronotropic cardiac activity, and positive inotropic effects.

Adult

0.3 mg (0.3 mL of 1:1000 epinephrine injection, USP) IM (EpiPen dose); may repeat q15min prn; inject into anterolateral aspect of thigh

Pediatric

0.01 mg/kg (0.01 mL/kg/dose of 1:1000 solution) IM; not to exceed 0.3 mg/dose (0.3 mL/dose)
EpiPen Jr dose is 0.15 mg IM to anterolateral thigh and may be appropriate for children 15-30 kg; if lesser dose of epinephrine needed, use another form of injectable epinephrine (not EpiPen Jr)

Increases toxicity of beta- and alpha-blocking agents and that of halogenated inhalational anesthetics

Documented hypersensitivity; cardiac arrhythmias; angle-closure glaucoma; during labor (may delay second stage of labor)

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Caution in elderly patients, prostatic hypertrophy, hypertension, cardiovascular disease, diabetes mellitus, hyperthyroidism, and cerebrovascular insufficiency; rapid IV infusions may cause death from cerebrovascular hemorrhage or cardiac arrhythmias

More on Mastocytosis

Overview: Mastocytosis
Differential Diagnoses & Workup: Mastocytosis
Treatment & Medication: Mastocytosis
Follow-up: Mastocytosis
Multimedia: Mastocytosis
References
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References

  1. Valent P. Diagnostic evaluation and classification of mastocytosis. Immunol Allergy Clin North Am. Aug 2006;26(3):515-34. [Medline].

  2. Patnaik MM, Rindos M, Kouides PA, Tefferi A, Pardanani A. Systemic mastocytosis: a concise clinical and laboratory review. Arch Pathol Lab Med. May 2007;131(5):784-91. [Medline].

  3. Horny HP, Sotlar K, Valent P. Mastocytosis: state of the art. Pathobiology. 2007;74(2):121-32. [Medline].

  4. Bussmann C, Hagemann T, Hanfland J, Haidl G, Bieber T, Novak N. Flushing and increase of serum tryptase after mechanical irritation of a solitary mastocytoma. Eur J Dermatol. Jul-Aug 2007;17(4):332-4. [Medline].

  5. Hartmann K, Artuc M, Baldus SE, et al. Expression of Bcl-2 and Bcl-xL in cutaneous and bone marrow lesions of mastocytosis. Am J Pathol. Sep 2003;163(3):819-26. [Medline].

  6. Noack F, Escribano L, Sotlar K, et al. Evolution of urticaria pigmentosa into indolent systemic mastocytosis: abnormal immunophenotype of mast cells without evidence of c-kit mutation ASP-816-VAL. Leuk Lymphoma. Feb 2003;44(2):313-9. [Medline].

  7. Brockow K, Akin C, Huber M, Metcalfe DD. IL-6 levels predict disease variant and extent of organ involvement in patients with mastocytosis. Clin Immunol. May 2005;115(2):216-23. [Medline].

  8. Brockow K, Jofer C, Behrendt H, Ring J. Anaphylaxis in patients with mastocytosis: a study on history, clinical features and risk factors in 120 patients. Allergy. Feb 2008;63(2):226-32. [Medline].

  9. Stein JA, Kamino H, Walters RF, Pomeranz MK. Mastocytosis with urticaria pigmentosa and osteoporosis. Dermatol Online J. Oct 15 2008;14(10):2. [Medline].

  10. Xu X, Solky B, Elenitsas R, Cotsarelis G. Scarring alopecia associated with mastocytosis. J Cutan Pathol. Oct 2003;30(9):561-5. [Medline].

  11. Kirsch R, Geboes K, Shepherd NA, et al. Systemic mastocytosis involving the gastrointestinal tract: clinicopathologic and molecular study of five cases. Mod Pathol. Dec 2008;21(12):1508-16. [Medline].

  12. Lappe U, Aumann V, Mittler U, Gollnick H. Familial urticaria pigmentosa associated with thrombocytosis as the initial symptom of systemic mastocytosis and Down's syndrome. J Eur Acad Dermatol Venereol. Nov 2003;17(6):718-22. [Medline].

  13. Soilleux EJ, Brown VL, Bowling J. Cutaneous mastocytosis localized to a radiotherapy field. Clin Exp Dermatol. Jan 2009;34(1):111-2. [Medline].

  14. Soilleux EJ, Grills C, Cooper SM. Cutaneous mastocytosis in human immunodeficiency virus: an unfortunate coincidence?. Clin Exp Dermatol. Aug 2008;33(5):619-21. [Medline].

  15. Matsumoto M, Ikeda M, Takeya M, Kodama H. Plane xanthoma associated with multiple mastocytoma. Pediatr Dermatol. Sep-Oct 2007;24(5):E66-9. [Medline].

  16. Butterfield JH. Systemic mastocytosis: clinical manifestations and differential diagnosis. Immunol Allergy Clin North Am. Aug 2006;26(3):487-513. [Medline].

  17. Schwartz LB. Diagnostic value of tryptase in anaphylaxis and mastocytosis. Immunol Allergy Clin North Am. Aug 2006;26(3):451-63. [Medline].

  18. Heide R, Beishuizen A, De Groot H, et al. Mastocytosis in children: a protocol for management. Pediatr Dermatol. Jul-Aug 2008;25(4):493-500. [Medline].

  19. Van Gysel D, Oranje AP, Vermeiden I, de Lijster de Raadt J, Mulder PG, van Toorenenbergen AW. Value of urinary N-methylhistamine measurements in childhood mastocytosis. J Am Acad Dermatol. Oct 1996;35(4):556-8. [Medline].

  20. Jordon RE. Immunologic Diseases of the Skin. East Norwalk, Conn: Appleton & Lange; 1991:245-8.

  21. Verma KK, Bhat R, Singh MK. Bullous mastocytosis treated with oral betamethasone therapy. Indian J Pediatr. Mar 2004;71(3):261-3. [Medline].

  22. Kinsler VA, Hawk JL, Atherton DJ. Diffuse cutaneous mastocytosis treated with psoralen photochemotherapy: case report and review of the literature. Br J Dermatol. Jan 2005;152(1):179-80. [Medline].

  23. Gobello T, Mazzanti C, Sordi D, et al. Medium- versus high-dose ultraviolet A1 therapy for urticaria pigmentosa: a pilot study. J Am Acad Dermatol. Oct 2003;49(4):679-84. [Medline].

  24. [Guideline] Joint Task Force on Practice Parameters; American Academy of Allergy, Asthma and Immunology; American College of Allergy, Asthma and Immunology; Joint Council of Allergy, Asthma and Immunology. The diagnosis and management of anaphylaxis: an updated practice parameter. J Allergy Clin Immunol. Mar 2005;115(3 Suppl 2):S483-523. [Medline].

  25. Wilson TM, Metcalfe DD, Robyn J. Treatment of systemic mastocytosis. Immunol Allergy Clin North Am. Aug 2006;26(3):549-73. [Medline].

  26. Krishnan K, Ramu V, Krishnaswamy G, Vardhana H. Mastocytosis, Systemic. eMedicine from WebMD [serial online]. December 4, 2008;Available at http://emedicine.medscape.com/article/203948-overview.

  27. Hoffmann KM, Moser A, Lohse P, et al. Successful treatment of progressive cutaneous mastocytosis with imatinib in a 2-year-old boy carrying a somatic KIT mutation. Blood. Sep 1 2008;112(5):1655-7. [Medline].

  28. Siebenhaar F, Kuhn W, Zuberbier T, Maurer M. Successful treatment of cutaneous mastocytosis and Ménière disease with anti-IgE therapy. J Allergy Clin Immunol. Jul 2007;120(1):213-5. [Medline].

  29. Ellis DL. Treatment of telangiectasia macularis eruptiva perstans with the 585-nm flashlamp-pumped dye laser. Dermatol Surg. Jan 1996;22(1):33-7. [Medline].

  30. Resh B, Jones E, Glaser DA. The cosmetic treatment of urticaria pigmentosa with Nd:YAG laser at 532 nanometers. J Cosmet Dermatol. Jun 2005;4(2):78-82. [Medline].

  31. Vlieg-Boerstra BJ, van der Heide S, Oude Elberink JN, Kluin-Nelemans JC, Dubois AE. Mastocytosis and adverse reactions to biogenic amines and histamine-releasing foods: what is the evidence?. Neth J Med. Jul-Aug 2005;63(7):244-9. [Medline].

  32. Allison MA, Schmidt CP. Reactivated urticaria pigmentosa. Cutis. Jul 1998;62(1):47-8. [Medline].

  33. Braverman IM. Skin Signs of Systemic Disease. 3rd ed. Philadelphia, Pa: WB Saunders; 1998:341-3.

  34. Brockow K, Scott LM, Worobec AS, et al. Regression of urticaria pigmentosa in adult patients with systemic mastocytosis: correlation with clinical patterns of disease. Arch Dermatol. Jun 2002;138(6):785-90. [Medline].

  35. Eichenfield LF, Honig PJ. Blistering disorders in childhood. Pediatr Clin North Am. Aug 1991;38(4):959-76. [Medline].

  36. Emanuel PD, Barton JC, Gualtieri RJ, Sams WM Jr. Urticaria pigmentosa and preleukemia: evidence for reactive mast cell proliferation. J Am Acad Dermatol. May 1991;24(5 Pt 2):893-7. [Medline].

  37. Fitzpatrick TB, Johnson RA, Wolff K. Color Atlas and Synopsis of Clinical Dermatology. 3rd ed. New York, NY: McGraw-Hill; 1997:562-5.

  38. Freedberg IM, Eisen AZ, Wolff K, eds. Fitzpatrick's Dermatology in General Medicine. 5th ed. New York, NY: McGraw-Hill; 1999:1902-8.

  39. Habif TP. Clinical Dermatology: A Color Guide to Diagnosis and Therapy. 3rd ed. Philadelphia, Pa: Mosby; 1996:144-5.

  40. Hartmann K, Henz BM. Mastocytosis: recent advances in defining the disease. Br J Dermatol. Apr 2001;144(4):682-95. [Medline].

  41. Has C, Misery L, David L, Cambazard F. Recurring staphylococcal scalded skin syndrome-like bullous mastocytosis: the utility of cytodiagnosis and the rapid regression with steroids. Pediatr Dermatol. May-Jun 2002;19(3):220-3. [Medline].

  42. Kastrup EK. Drug Facts and Comparisons. St. Louis, Mo: Facts and Comparisons; 1998:1153-6, 1597-1600, 2057-67.

  43. Leaf FA, Jaecks EP, Rodriguez DR. Bullous urticaria pigmentosa. Cutis. Nov 1996;58(5):358-60. [Medline].

  44. Lever WF. Histopathology of the Skin. 8th ed. Philadelphia, Pa: Lippincott-Raven; 1997:141-3.

  45. Longley J, Duffy TP, Kohn S. The mast cell and mast cell disease. J Am Acad Dermatol. Apr 1995;32(4):545-61; quiz 562-4. [Medline].

  46. Roberts LJ 2nd, Sweetman BJ, Lewis RA, Austen KF, Oates JA. Increased production of prostaglandin D2 in patients with systemic mastocytosis. N Engl J Med. Dec 11 1980;303(24):1400-4. [Medline].

  47. Schachner LA, Hansen RC. Pediatric Dermatology. 2nd ed. New York, NY: Churchill Livingstone; 1995:852-8.

  48. Soter NA. The skin in mastocytosis. J Invest Dermatol. Mar 1991;96(3):32S-38S; discussion 38S-39S. [Medline].

  49. van Toorenenbergen AW, Oranje AP. Comparison of serum tryptase and urine N-methylhistamine in patients with suspected mastocytosis. Clin Chim Acta. Sep 2005;359(1-2):72-7. [Medline].

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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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