Mastocytosis is a heterogenic disease characterized by a pathological increase of mast cells in different organs, particularly in the skin and bone marrow. The disease categories range from benign cutaneous mastocytosis (CM) to progressive forms with organ dysfunction. Most adult patients have indolent systemic mastocytosis (ISM) associated with maculopapular cutaneous lesions and are suffering from symptoms resulting from mast cell mediator release, include whealing, erythema and pruritus after mechanical irritation of skin lesions (Darier’s sign), but also flushing, headaches, bone pain, nausea, vomiting, diarrhea, abdominal pain, up to severe and life-threatening anaphylaxis. Therefore, many patients require continuous and emergency medications. As of today, antihistamines are the first-line therapeutic strategy and are being routinely used in the treatment of mastocytosis also in combination with additional mediator blocking agents like H2-blockers or leukotriene antagonists. However, these treatment strategies in most patients are still insufficient to control symptoms. Recent therapeutic developments focus on the reduction of mast cell burden in more aggressive forms of mastocytosis, whereas further-reaching treatment options targeting mediator release by mast cells or better control of mediator-related symptoms in indolent mastocytosis are missing. Therefore, this EAACI Task force aims to increase awareness of mediator-related symptoms in cutaneous and indolent systemic mastocytosis in the field of dermatological allergology and clinical immunology and to identify and define the unmet needs for the development of novel therapeutic options. The Task force will establish an expert consortium to review the literature, discuss current and possible future developments and create a position paper on the unmet needs in the treatment of cutaneous and indolent systemic mastocytosis.
Last updated 07 November 2014