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
- Horny HP, Metcalfe DD, Bennet JM, et al. Mastocytosis. In: WHO Classification of the Tumors of Haematopoietic and Lymphoid Tissues, Swerdlow SH, Campo E, Harris NL, et al. (Eds), IARC, Lyon 2008. p.54.
- Alvarez-Twose I, Vañó-Galván S, Sánchez-Muñoz L, et al. Increased serum baseline tryptase levels and extensive skin involvement are predictors for the severity of mast cell activation episodes in children with mastocytosis. Allergy 2012; 67:813.
- Barnes M, Van L, DeLong L, Lawley LP. Severity of cutaneous findings predict the presence of systemic symptoms in pediatric maculopapular cutaneous mastocytosis. Pediatr Dermatol 2014; 31:271.
- Deverrière G, Carré D, Nae I, et al. [Bullous mastocytosis in infancy: a rare presentation]. Arch Pediatr 2012; 19:722.
- Hepner DL, Castells MC. Anaphylaxis during the perioperative period. Anesth Analg 2003; 97:1381.
- Carter MC, Uzzaman A, Scott LM, et al. Pediatric mastocytosis: routine anesthetic management for a complex disease. Anesth Analg 2008; 107:422.
- Ahmad N, Evans P, Lloyd-Thomas AR. Anesthesia in children with mastocytosis--a case based review. Paediatr Anaesth 2009; 19:97.
- Konrad FM, Unertl KE, Schroeder TH. [Mastocytosis. A challenge in anaesthesiology]. Anaesthesist 2009; 58:1239.
- Brockow K, Akin C, Huber M, Metcalfe DD. Assessment of the extent of cutaneous involvement in children and adults with mastocytosis: relationship to symptomatology, tryptase levels, and bone marrow pathology. J Am Acad Dermatol 2003; 48:508.
- Müller U, Helbling A, Hunziker T, et al. Mastocytosis and atopy: a study of 33 patients with urticaria pigmentosa. Allergy 1990; 45:597.
- Greenhawt M, Akin C. Mastocytosis and allergy. Curr Opin Allergy Clin Immunol 2007; 7:387.
- González de Olano D, Alvarez-Twose I, Esteban-López MI, et al. Safety and effectiveness of immunotherapy in patients with indolent systemic mastocytosis presenting with Hymenoptera venom anaphylaxis. J Allergy Clin Immunol 2008; 121:519.
- Ruëff F, Przybilla B, Biló MB, et al. Predictors of severe systemic anaphylactic reactions in patients with Hymenoptera venom allergy: importance of baseline serum tryptase-a study of the European Academy of Allergology and Clinical Immunology Interest Group on Insect Venom Hypersensitivity. J Allergy Clin Immunol 2009; 124:1047.
- Hartmann K, Metcalfe DD. Pediatric mastocytosis. Hematol Oncol Clin North Am 2000; 14:625.
- Worobec AS. Treatment of systemic mast cell disorders. Hematol Oncol Clin North Am 2000; 14:659.
- Póvoa P, Ducla-Soares J, Fernandes A, Palma-Carlos AG. A case of systemic mastocytosis; therapeutic efficacy of ketotifen. J Intern Med 1991; 229:475.
- Kettelhut BV, Berkebile C, Bradley D, Metcalfe DD. A double-blind, placebo-controlled, crossover trial of ketotifen versus hydroxyzine in the treatment of pediatric mastocytosis. J Allergy Clin Immunol 1989; 83:866.
- Escribano L. Section Chief, Hematology Services, Director, Mast Cell Unit, Hospital Ramon y Cajal, Madrid, personal communication.
- Moore C, Ehlayel MS, Junprasert J, Sorensen RU. Topical sodium cromoglycate in the treatment of moderate-to-severe atopic dermatitis. Ann Allergy Asthma Immunol 1998; 81:452.
- Stainer R, Matthews S, Arshad SH, et al. Efficacy and acceptability of a new topical skin lotion of sodium cromoglicate (Altoderm) in atopic dermatitis in children aged 2-12 years: a double-blind, randomized, placebo-controlled trial. Br J Dermatol 2005; 152:334.
- Heide R, Beishuizen A, De Groot H, et al. Mastocytosis in children: a protocol for management. Pediatr Dermatol 2008; 25:493.
- Godt O, Proksch E, Streit V, Christophers E. Short- and long-term effectiveness of oral and bath PUVA therapy in urticaria pigmentosa and systemic mastocytosis. Dermatology 1997; 195:35.
- Sotiriou E, Apalla Z, Ioannides D. Telangiectasia macularis eruptive perstans successfully treated with PUVA therapy. Photodermatol Photoimmunol Photomed 2010; 26:46.
- Rishpon A, Matz H, Gat A, Brenner S. Telangiectasia macularis eruptiva perstans: unusual presentation and treatment. Skinmed 2006; 5:300.
- Brazzelli V, Grasso V, Manna G, et al. Indolent systemic mastocytosis treated with narrow-band UVB phototherapy: study of five cases. J Eur Acad Dermatol Venereol 2012; 26:465.
- Study identifier NCT01920204. www.clinicaltrials.gov (Accessed on September 26, 2014).
- JOHNSON WC, HELWIG EB. Solitary mastocytosis (urticaria pigmentosa). Arch Dermatol 1961; 84:806.
- Uzzaman A, Maric I, Noel P, et al. Pediatric-onset mastocytosis: a long term clinical follow-up and correlation with bone marrow histopathology. Pediatr Blood Cancer 2009; 53:629.
- Méni C, Bruneau J, Georgin-Lavialle S, et al. Paediatric mastocytosis: a systematic review of 1747 cases. Br J Dermatol 2015; 172:642.
- Auquit-Auckbur I, Lazar C, Deneuve S, et al. Malignant transformation of mastocytoma developed on skin mastocytosis into cutaneous mast cell sarcoma. Am J Surg Pathol 2012; 36:779.
- Chantorn R, Shwayder T. Death from mast cell leukemia: a young patient with longstanding cutaneous mastocytosis evolving into fatal mast cell leukemia. Pediatr Dermatol 2012; 29:605.
- 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