Cutaneous mastocytosis treatment: strategies, limitations and perspectives

Abstract

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.

Molecular Background, Clinical Features and Management of Pediatric Mastocytosis: Status 2021

“…Parents and caregivers of children with mastocytosis should be informed about environmental factors that can cause symptoms through the release of MC mediators [ 13 , 135 , 136 , 137 , 138 , 139 ]. Triggers vary greatly from patient to patient, and include mechanical or other physical stimuli, infections, teething, allergens, and drugs [ 7 , 12 , 90 , 106 , 107 , 108 , 109 , 110 , 125 , 126 , 135 , 136 , 137 , 138 , 139 , 140 ] ( Table 3 ). Simple measures such as avoiding friction on affected skin area, heat exposure and sudden temperature changes, can have a significant impact on mitigating symptoms and preventing exacerbations [ 101 , 136 , 137 ].…”

“…Various drugs have been considered as potential triggers of MC activation. These include, among others, NSAIDs, opioids, muscle relaxants, quinolones, succinylcholine, agents with tetrahydroisiquinoline, and cough suppressants [ 123 , 132 , 135 , 137 , 139 , 140 ]. However, significant reactions to these agents are rare.…”

“…In children, the decision to prescribe an “Epi-Pen” is based on symptoms, course, and subtype of CM. Current recommendations suggest to provide the Epi-Pen to children with extensive skin lesions, a history of severe systemic symptoms or anaphylaxis, and highly elevated serum tryptase levels [ 108 , 131 , 132 , 135 , 136 , 137 , 138 , 139 ]. In case of severe anaphylaxis, epinephrine is dosed by body weight at 0.01 mg per kg of body weight to a maximum dose of 0.5 mg.…”

Urticária pigmentosa no adulto: tratamento bem-sucedido em associação com fototerapia

“…Na MS, podem ser usadas drogas citorredutoras. Atualmente, os imunobiológicos omalizumabe e imatinibe vêm sendo estudados em casos graves com anafilaxia e doença sistêmica 4,5 .…”

Cutaneous mastocytosis: A dermatological perspective

“…In extensive urticaria pigmentosa and diffuse CM, both oral psoralen with ultraviolet light (PUVA), and natural sunlight have been shown to be effective 45 . Second‐line treatment options for more aggressive forms of CM are imatinib, a tyrosine kinase inhibitor and omalizumab, a humanised IgG1 monoclonal antibody against IgE 46,47,48 …”