eISSN: 2299-0046
ISSN: 1642-395X
Advances in Dermatology and Allergology/Postępy Dermatologii i Alergologii
Current issue Archive Manuscripts accepted About the journal Abstracting and indexing Subscription Contact Instructions for authors
SCImago Journal & Country Rank
vol. 35
Review paper

Cutaneous mastocytosis treatment: strategies, limitations and perspectives

Justyna Czarny, Magdalena Lange, Hanna Ługowska-Umer, Roman J. Nowicki

Adv Dermatol Allergol 2018; XXXV (6): 541-545
Online publish date: 2018/08/13
View full text
Get citation
JabRef, Mendeley
Papers, Reference Manager, RefWorks, Zotero
Mastocytosis is a rare myeloproliferative disease, characterized by excessive proliferation and accumulation of mast cells in the tissues. In cutaneous mastocytosis (CM), mast cells infiltration is limited to the skin, whereas in systemic mastocytosis (SM) internal organs are involved. The first-line treatment in CM is antimediator therapy (mainly H1 and H2 antihistamines) and short-term topical corticosteroids. Phototherapy is the second-line therapy which may be considered when antihistamines do not produce the expected improvement. New therapeutic options include omalizumab and KIT-targeting agents. Although the disappearance of skin lesions has been reported as a result of cytoreductive therapies in SM, the use of potentially toxic drugs in CM is not recommended. In all adults with mastocytosis and in pediatric patients with severe CM, a persistently elevated serum tryptase level and anaphylaxis in medical history, equipping with epinephrine autoinjector for use in case of anaphylaxis is recommended.

mastocytosis, treatment, prophylaxis

Hartmann K, Escribano L, Grattan C, et al. Cutaneous manifestations in patients with mastocytosis: Consensus report of the European Competence Network on Mastocytosis; the American Academy of Allergy, Asthma & Immunology; and the European Academy of Allergology and Clinical Immunology. J Allergy Clin Immunol 2016; 137: 35-45.
Pardanani A. Systemic mastocytosis in adults: 2017 update on diagnosis, risk stratification, and management. Am J Hematol 2016; 91: 1146-59.
Valent P, Akin C, Metcalfe DD. Mastocytosis: 2016 updated WHO classification and novel emerging treatment concepts. Blood 2017; 129: 1420-7.
Siebenhaar F, Akin C, Bindslev-Jensen C, et al. Treatment strategies in mastocytosis. Immunol Allergy Clin North Am 2014; 34: 433-47.
Arock M, Akin C, Hermine O, et al. Current treatment options in patients with mastocytosis: status in 2015 and future perspectives. Eur J Haematol 2015; 94: 474-90.
Azańa JM, Torrelo A, Matito A. Update on mastocytosis
(part 2): categories, prognosis, and treatment. Actas Dermosifiliogr 2016; 107: 15-22.
Broesby-Olsen S, Dybedal I, Gülen T, et al. Multidisciplinary management of mastocytosis: Nordic Expert Group Consensus. Acta Derm Venereol 2016; 96: 602-12.
Zuberbier T, Aberer W, Asero R, et al. Methods report on the development of the 2013 revision and update of the EAACI/GA2 LEN/EDF/WAO guideline for the definition, classification, diagnosis, and management of urticaria. Allergy 2014; 69: e1-29.
Siebenhaar F, Förtsch A, Krause K, et al. Rupatadine improves quality of life in mastocytosis: a randomized, double-blind, placebo-controlled trial. Allergy 2013; 68: 949-52.
Verma KK, Bhat R, Singh MK. Bullous mastocytosis treated with oral betamethasone therapy. Indian J Pediatr 2004; 71: 261-3.
Yankova R, Abadjieva T, Belovezhdov V. Cutaneous mastocytosis with persistent blistering: successful treatment with methylprednisolone and 3-year follow-up management. Dermatol Ther (Heidelb) 2015; 5: 145-50.
Brazzelli V, Grassi S, Merante S, et al. Narrow-band UVB phototherapy and psoralen-ultraviolet A photochemotherapy in the treatment of cutaneous mastocytosis: a study in 20 patients. Photodermatol Photoimmunol Photomed 2016; 32: 238-46.
Guhl S, Hartmann K, Tapkenhinrichs S, et al. Ultraviolet irradiation induces apoptosis in human immature, but not in skin mast cells. J Invest Dermatol 2003; 121: 837-44.
Resh B, Jones E, Glaser DA. The cosmetic treatment of urticaria pigmentosa with Nd:YAG laser at 532 nanometers. J Cosmet Dermatol 2005; 4: 78-82.
Correia O, Duarte AF, Quirino P, et al. Cutaneous mastocytosis: two pediatric cases treated with topical pimecrolimus. Dermatol Online J 2010; 16: 8.
Sokol KC, Ghazi A, Kelly BC, et al. Omalizumab as a desensitizing agent and treatment in mastocytosis: a review of the literature and case report. J Allergy Clin Immunol Pract 2014; 2: 266-70.
Kibsgaard L, Skjold T, Deleuran M, et al. Omalizumab induced remission of idiopathic anaphylaxis in a patient suffering from indolent systemic mastocytosis. Acta Derm Venereol 2014; 94: 363-4.
Matito A, Blázquez-Gońi C, Morgado JM, et al. Short-term omalizumab treatment in an adolescent with cutaneous mastocytosis. Ann Allergy Asthma Immunol 2013; 111: 425-6.
Morren MA, Hoppé A, Renard M, et al. Imatinib mesylate in the treatment of diffuse cutaneous mastocytosis. J Pediatr 2013; 162: 205-7.
Vannorsdall EJ, Collins JA, Chen QC, et al. Symptomatic response to imatinib mesylate in cutaneous mastocytosis associated with chronic myelomonocytic leukemia. Curr Oncol 2013; 20: 349-53.
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 2008; 112: 1655-7.
Lortholary O, Chandesris MO, Bulai Livideanu C, et al. Masitinib for treatment of severely symptomatic indolent systemic mastocytosis: a randomised, placebo-controlled, phase 3 study. Lancet 2017; 389: 612-20.
Paul C, Sans B, Suarez F, et al. Masitinib for the treatment of systemic and cutaneous mastocytosis with handicap: a phase 2a study. Am J Hematol 2010; 85: 921-5.
Lim KH, Pardanani A, Butterfield JH, et al. Cytoreductive therapy in 108 adults with systemic mastocytosis: outcome analysis and response prediction during treatment with interferon-alpha, hydroxyurea, imatinib mesylate or 2-chlorodeoxyadenosine. Am J Hematol 2009; 84: 790-4.
Barete S, Lortholary O, Damaj G, et al. Long-term efficacy and safety of cladribine (2-CdA) in adult patients with mastocytosis. Blood 2015; 126: 1009-16.
Lock AD, McNamara CJ, Rustin MH. Sustained improvement in urticaria pigmentosa and pruritus in a case of indolent systemic mastocytosis treated with cladribine. Clin Exp Dermatol 2015; 40: 142-5.
Casassus P, Caillat-Vigneron N, Martin A, et al. Treatment of adult systemic mastocytosis with interferon-alpha: results of a multicentre phase II trial on 20 patients. Br J Haematol 2002; 119: 1090-7.
Rosario A, Bhat RM. Successful treatment of provisional cutaneous mastocytosis with interferon alpha. Indian J Dermatol 2016; 61: 347.
Hartmann K, Siebenhaar F, Belloni B, et al. Effects of topical treatment with the raft modulator miltefosine and clobetasol in cutaneous mastocytosis: a randomized, double-blind, placebo-controlled trial. Br J Dermatol 2010; 162: 185-90.
Górska A, Niedoszytko M, Lange M, et al. Risk factors for anaphylaxis in patients with mastocytosis. Pol Arch Med Wewn 2015; 125: 46-53.
Brockow K, Jofer C, Behrendt H, et al. Anaphylaxis in patients with mastocytosis: a study on history, clinical features and risk factors in 120 patients. Allergy 2008; 63: 226-32.
Muraro A, Roberts G, Worm M, et al. Anaphylaxis: guidelines from the European Academy of Allergy and Clinical Immunology. Allergy 2014; 69: 1026-45.
Matito A, Morgado JM, Sánchez-López P, et al. Management of anesthesia in adult and pediatric mastocytosis: a study of the Spanish Network on Mastocytosis (REMA) based on 726 anesthetic procedures. Int Arch Allergy Immunol 2015; 167: 47-56.
Méni C, Bruneau J, Georgin-Lavialle S, et al. Paediatric mastocytosis: a systematic review of 1747 cases. Br J Dermatol 2015; 172: 642-51.
González de Olano D, de la Hoz Caballer B, Núńez López R, et al. Prevalence of allergy and anaphylactic symptoms in 210 adult and pediatric patients with mastocytosis in Spain: a study of the Spanish network on mastocytosis (REMA). Clin Exp Allergy 2007; 37: 1547-55.
Matito A, Carter M. Cutaneous and systemic mastocytosis in children: a risk factor for anaphylaxis? Curr Allergy Asthma Rep 2015; 15: 22.
Lange M, Niedoszytko M, Renke J, et al. Clinical aspects of paediatric mastocytosis: a review of 101 cases. J Eur Acad Dermatol Venereol 2013; 27: 97-102.
Czarny JH, Lange M, Niedoszytko MB, et al. Drug hypersensitivity reactions and allergy in patients with mastocytosis. Przegl Dermatol 2017; 104: 22-30.
Brockow K, Bonadonna P. Drug allergy in mast cell disease. Curr Opin Allergy Clin Immunol 2012; 12: 354-60.
Bonadonna P, Lombardo C. Drug allergy in mastocytosis. Immunol Allergy Clin North Am 2014; 34: 397-405.
Dewachter P, Castells MC, Hepner DL, et al. Mouton-Faivre C. Perioperative management of patients with mastocytosis. Anesthesiology 2014; 120: 753-9.
Bonadonna P, Zanotti R, Muller U. Mastocytosis and insect venom allergy. Curr Opin Allergy Clin Immunol 2010; 10: 347-53.
Niedoszytko M, de Monchy J, van Doormaal JJ, et al. Mastocytosis and insect venom allergy: diagnosis, safety and efficacy of venom immunotherapy. Allergy 2009; 64: 1237-45.
Quick links
© 2019 Termedia Sp. z o.o. All rights reserved.
Developed by Bentus.
PayU - płatności internetowe