Table of ContentsView AllTable of ContentsWhen to TreatTreating the Skin With ISMOther Treatments for ISMManaging SymptomsSide Effect Treatments
Table of ContentsView All
View All
Table of Contents
When to Treat
Treating the Skin With ISM
Other Treatments for ISM
Managing Symptoms
Side Effect Treatments
As its name implies, “indolent” systemic mastocytosis progresses slowly and does not present an immediate threat. Treatment for this form of the disease aims to identify and avoid symptom triggers.
This disease causes excess abnormal mast cells that build up in organ tissue. When a trigger arises, these cells overreact and release too much of a chemical mediator, such ashistamine, to fight off a perceived threat. The effect causes symptoms like an allergic reaction in the organs involved. Treatment mostly includes self-management and certain drugs.
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When to Treat Indolent Systemic Mastocytosis
Indolent systemic mastocytosis has a better prognosis than other forms of mastocytosis. The disease typically spares organs from damage and rarely progresses to the more severe forms. People with this condition generally live a normal lifespan, comparable to their peers.
However, it can be highly symptomatic. In addition to physical symptoms, psychological and neurological symptoms that lead to reduced performance can also occur. Symptoms that interfere with physical and or emotional well-being warrant treatment.
Even if symptoms are minor and medical treatment is not needed, guidelines from the National Comprehensive Cancer Network (NCCN) advise that people with indolent systemic mastocytosis should receive the following:
Emergency Treatment for Life-Threatening Symptoms of ISMYou have a higher risk of symptoms of anaphylaxis (a severe and potentially life-threatening allergic reaction) if you have any form of systemic mastocytosis. This medical emergency can occur when histamine levels in your blood quickly and intensely increase due to an abnormally high number ofmast cellsoverreacting to a trigger.Symptoms of anaphylaxis can occur within seconds to minutes of exposure to a trigger. They require immediate treatment with an EpiPen and a visit to a hospital emergency room. Common signs of anaphylaxis include:Abdominal pain,nausea, orvomitingChest tightnessDizzinessorsyncope(fainting)Feeling of doomHypotension(low blood pressure)Swelling of the mouth, throat, and airwaysTachycardia(rapid heartbeat)Throat hoarseness or tightnessTrouble breathingUrticaria(hives)Welts, or red, itchy skin
Emergency Treatment for Life-Threatening Symptoms of ISM
You have a higher risk of symptoms of anaphylaxis (a severe and potentially life-threatening allergic reaction) if you have any form of systemic mastocytosis. This medical emergency can occur when histamine levels in your blood quickly and intensely increase due to an abnormally high number ofmast cellsoverreacting to a trigger.Symptoms of anaphylaxis can occur within seconds to minutes of exposure to a trigger. They require immediate treatment with an EpiPen and a visit to a hospital emergency room. Common signs of anaphylaxis include:Abdominal pain,nausea, orvomitingChest tightnessDizzinessorsyncope(fainting)Feeling of doomHypotension(low blood pressure)Swelling of the mouth, throat, and airwaysTachycardia(rapid heartbeat)Throat hoarseness or tightnessTrouble breathingUrticaria(hives)Welts, or red, itchy skin
You have a higher risk of symptoms of anaphylaxis (a severe and potentially life-threatening allergic reaction) if you have any form of systemic mastocytosis. This medical emergency can occur when histamine levels in your blood quickly and intensely increase due to an abnormally high number ofmast cellsoverreacting to a trigger.
Symptoms of anaphylaxis can occur within seconds to minutes of exposure to a trigger. They require immediate treatment with an EpiPen and a visit to a hospital emergency room. Common signs of anaphylaxis include:
Treatment for Indolent Systemic Mastocytosis Limited to Skin
There is no single treatment for indolent systemic mastocytosis limited to the skin. The specific treatment you receive will depend on many factors, including the extent and severity of your disease, your age, medical history, and other health conditions.
First-Line Therapy
First-generation and second-generation H1 (histamine 1)antihistaminesare the cornerstone of first-line therapy for indolent systemic mastocytosis limited to the skin. These drugs often succeed in relieving symptoms ofpruritus(itching),facial flushing, and burning of the skin.
Common first-generation H1 antihistamines include:
Second-generation antihistamines may tend to cause less drowsiness than first-generation drugs. Common second-generation H1 antihistamines include:
For persistent symptoms, treatment may be expanded to include the following medications:
H2 (histamine 2) antihistamines:
Leukotrieneinhibitors:
Other Therapies:
Second-Line Therapy:
Second-line therapy is used for skin symptoms that are not responsive to first-line therapy:
Treatment for Indolent Systemic Mastocytosis
There is no single treatment plan for indolent systemic mastocytosis because this disease affects everyone differently. Treatment for this disorder is mostly based on expert opinion rather than evidence from controlled clinical trials. This is partly because it is a heterogeneous disease that can affect a combination of body systems, but only involves about 1 per 10,000 people.
Indolent systemic mastocytosis involves at least one organ system other than the skin. Since few cases progress to advanced forms of the disease, treatment focuses on improving and preventing symptoms related to mediators such as histamine. Other treatments may be used for more advanced forms of the disease.
Some common treatments for indolent systemic mastocytosis include:
Neuropsychiatric System
The following treatments may help with brain fog:
Cardiovascular Systemincludes the use of EpiPens, which treat low blood pressure that may be spontaneous or observed after an insect bite or other trigger.
Respiratory System
The following treatments can help with respiratory symptoms such as wheezing and shortness of breath:
Leukotriene inhibitors:
Musculoskeletal System
The following treatments center on treating symptoms ofosteoporosis(loss of bone density) and bone pain:
Digestive System
The following medications may be helpful in treating gastrointestinal symptoms:
Whole-Body System
The following treatments can provide benefits for your entire body:
ISM Alternative/Herbal TreatmentsWhile traditional medicine can help manage indolent systemic mastocytosis, some people report success with alternative or herbal treatments. Though scientific research is limited, there is anecdotal evidence that consuming the following anti-inflammatory foods and herbs may help symptoms of the disease::Brazil nutsChamomile teaFiberGalangal (Thai ginger)Moringa (horseradish tree)Nettle teaOnionsPeachesTurmeric
ISM Alternative/Herbal Treatments
While traditional medicine can help manage indolent systemic mastocytosis, some people report success with alternative or herbal treatments. Though scientific research is limited, there is anecdotal evidence that consuming the following anti-inflammatory foods and herbs may help symptoms of the disease::Brazil nutsChamomile teaFiberGalangal (Thai ginger)Moringa (horseradish tree)Nettle teaOnionsPeachesTurmeric
While traditional medicine can help manage indolent systemic mastocytosis, some people report success with alternative or herbal treatments. Though scientific research is limited, there is anecdotal evidence that consuming the following anti-inflammatory foods and herbs may help symptoms of the disease::
Managing Symptoms and Secondary Conditions
Symptoms and secondary conditions of indolent systemic mastocytosis can range in severity due to the heterogeneous nature of the disease.
Many symptoms are episodic, becoming intense in unpredictable, recurrent flares that occur in response to triggers or for seemingly no reason at all. Since the disorder affects everyone differently, you may not known which organs will be affected or how you will react until symptoms begin.
In addition to treatments specific to symptoms, some people succeed in working to avoid known triggers at home, at work, and in their social lives. However, since triggers can range from temperature variations to the development of new allergies, avoiding triggers can often affect how you live your life.
High-histamine foods include those that are overly processed, fermented, or spoiled. Examples of high-histamine foods include:
Managing symptoms can also help reduce your risk of complications and secondary conditions. Common comorbidities (co-occurring conditions) with ISM include:
How to Treat ISM Treatment Side Effects
Limiting or avoiding ISM treatment side effects requires communicating with your care team so they can help you manage these issues. Finding the right treatment regimen can be a complex process.
Factors such as the rarity of the disease and a provider’s unfamiliarity with it can complicate treatment. In some cases, it can contribute to a delayed or hindered diagnosis, impacting side effects and treatment outcomes.
While an allergist orimmunologistmay help you get a proper diagnosis of indolent systemic mastocytosis, the NCCN advises that treatment of all types of mastocytosis ideally includes a multidisciplinary approach involving the following specialists:
This team of specialists works together to address side effects and treatment changes from all aspects of the disease.
Side effects of ISM treatment add to the unpredictability of the disease and the wide range of invisible and visible symptoms it can cause. While your healthcare team can help you manage many side effects of treatment, research indicates that people with systemic mastocytosis have also used the following strategies to face these challenges:
Summary
Indolent systemic mastocytosis can present with many types of symptoms. These can range from mostly skin-centered to systemic problems such as flushing and bone pain. The risk of anaphylaxis is also higher than normal.
Treatment for indolent systemic mastocytosis centers on easing mast cell mediators and their impact on affected organs. Most people with this disease can expect to live a normal lifespan with little risk of severe disease. However, symptoms need proper treatment to maintain comfort and a normal life.
Finding the right treatment can be challenging for this rare and heterogeneous disease. While an allergist or immunologist may manage your care, having a multidisciplinary treatment team is advised.
20 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Merck Manual Professional Version.Mastocytosis and mast cell activation syndrome.Buonomo A, Nucera E, Criscuolo M.Treatment of indolent and advanced systemic mastocytosis.Mediterr J Hematol Infect Dis. 2022 May 1;14(1):e2022040. doi:10.4084/MJHID.2022AB Science.Indolent systemic mastocytosis.Pardanani A.How I treat patients with indolent and smoldering mastocytosis (rare conditions but difficult to manage).Blood. 2013;121(16)3085-3094. doi:10.1182/blood-2013-01-453183UWHealth.Mast cell activation syndrome and diet.NHS.Overview: mastocytosis.Allergy and Asthma Network.What is anaphylaxis?Czarny J, Lange M, Ługowska-Umer H, Nowicki RJ.Cutaneous mastocytosis treatment: strategies, limitations and perspectives.Postepy Dermatol Alergol. 2018 Dec;35(6):541-545. doi:10.5114/ada.2018.77605The Mast Cell Disease Society.Medications to treat mast cell diseases.González-de-Olano D, Matito A, Orfao A, Escribano L.Advances in the understanding and clinical management of mastocytosis and clonal mast cell activation syndromes.F1000Res. 2016 Nov 14;5:2666. doi:10.12688/f1000research.9565.1Sciumè M, De Magistris C, Galli N, Ferretti E, Milesi G, De Roberto P, Fabris S, Grifoni FI.Target therapies for systemic mastocytosis: an update.Pharmaceuticals (Basel). 2022 Jun 11;15(6):738. doi:10.3390/ph15060738National Organization for Rare Disorders (NORD).Mastocytosis.ASCO Post (American Society of Clinical Oncology).FDA approves avapritinib for indolent systemic mastocytosis.Slapnicar C, Trinkaus M, Hicks L, Vadas P.Efficacy of omalizumab in indolent systemic mastocytosis.Case Rep Hematol. 2019 Sep 16;2019:3787586. doi:10.1155/2019/3787586The Institute for Functional Medicine.Fight inflammation and stabilize mast cells naturally.It’sSMthing: Systemic Mastocytosis.With many symptoms come many questions.National Comprehensive Cancer Network (NCCN).NCCN guidelines for patients: systemic mastocytosis.Mikkelsen CS, Nybo A, Arvesen KB, Holk-Poulsen J.Delayed diagnosis of adult indolent systemic mastocytosis.Dermatol Reports. 2014 Feb 17;6(1):5199. doi:10.4081/dr.2014.5199Gotlib J., Gerds, AT, Abdelmessieh, P, Ali, H, et al.NCCN guidelines insights: systemic mastocytosis, version 3.2024: featured updates to the NCCN guidelines.Journal of the National Comprehensive Cancer Network, 2024;22(2D)e240030. doi:10.6004/jnccn.2024.0030Nilsson A, Ungerstedt J, Hedström M. Living with systemic mastocytosis:Balancing between vulnerability and resilience: a qualitative study.European Journal of Oncology Nursing. October 2022;60;102172. doi:10.1016/j.ejon.2022.102172
20 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Merck Manual Professional Version.Mastocytosis and mast cell activation syndrome.Buonomo A, Nucera E, Criscuolo M.Treatment of indolent and advanced systemic mastocytosis.Mediterr J Hematol Infect Dis. 2022 May 1;14(1):e2022040. doi:10.4084/MJHID.2022AB Science.Indolent systemic mastocytosis.Pardanani A.How I treat patients with indolent and smoldering mastocytosis (rare conditions but difficult to manage).Blood. 2013;121(16)3085-3094. doi:10.1182/blood-2013-01-453183UWHealth.Mast cell activation syndrome and diet.NHS.Overview: mastocytosis.Allergy and Asthma Network.What is anaphylaxis?Czarny J, Lange M, Ługowska-Umer H, Nowicki RJ.Cutaneous mastocytosis treatment: strategies, limitations and perspectives.Postepy Dermatol Alergol. 2018 Dec;35(6):541-545. doi:10.5114/ada.2018.77605The Mast Cell Disease Society.Medications to treat mast cell diseases.González-de-Olano D, Matito A, Orfao A, Escribano L.Advances in the understanding and clinical management of mastocytosis and clonal mast cell activation syndromes.F1000Res. 2016 Nov 14;5:2666. doi:10.12688/f1000research.9565.1Sciumè M, De Magistris C, Galli N, Ferretti E, Milesi G, De Roberto P, Fabris S, Grifoni FI.Target therapies for systemic mastocytosis: an update.Pharmaceuticals (Basel). 2022 Jun 11;15(6):738. doi:10.3390/ph15060738National Organization for Rare Disorders (NORD).Mastocytosis.ASCO Post (American Society of Clinical Oncology).FDA approves avapritinib for indolent systemic mastocytosis.Slapnicar C, Trinkaus M, Hicks L, Vadas P.Efficacy of omalizumab in indolent systemic mastocytosis.Case Rep Hematol. 2019 Sep 16;2019:3787586. doi:10.1155/2019/3787586The Institute for Functional Medicine.Fight inflammation and stabilize mast cells naturally.It’sSMthing: Systemic Mastocytosis.With many symptoms come many questions.National Comprehensive Cancer Network (NCCN).NCCN guidelines for patients: systemic mastocytosis.Mikkelsen CS, Nybo A, Arvesen KB, Holk-Poulsen J.Delayed diagnosis of adult indolent systemic mastocytosis.Dermatol Reports. 2014 Feb 17;6(1):5199. doi:10.4081/dr.2014.5199Gotlib J., Gerds, AT, Abdelmessieh, P, Ali, H, et al.NCCN guidelines insights: systemic mastocytosis, version 3.2024: featured updates to the NCCN guidelines.Journal of the National Comprehensive Cancer Network, 2024;22(2D)e240030. doi:10.6004/jnccn.2024.0030Nilsson A, Ungerstedt J, Hedström M. Living with systemic mastocytosis:Balancing between vulnerability and resilience: a qualitative study.European Journal of Oncology Nursing. October 2022;60;102172. doi:10.1016/j.ejon.2022.102172
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Merck Manual Professional Version.Mastocytosis and mast cell activation syndrome.Buonomo A, Nucera E, Criscuolo M.Treatment of indolent and advanced systemic mastocytosis.Mediterr J Hematol Infect Dis. 2022 May 1;14(1):e2022040. doi:10.4084/MJHID.2022AB Science.Indolent systemic mastocytosis.Pardanani A.How I treat patients with indolent and smoldering mastocytosis (rare conditions but difficult to manage).Blood. 2013;121(16)3085-3094. doi:10.1182/blood-2013-01-453183UWHealth.Mast cell activation syndrome and diet.NHS.Overview: mastocytosis.Allergy and Asthma Network.What is anaphylaxis?Czarny J, Lange M, Ługowska-Umer H, Nowicki RJ.Cutaneous mastocytosis treatment: strategies, limitations and perspectives.Postepy Dermatol Alergol. 2018 Dec;35(6):541-545. doi:10.5114/ada.2018.77605The Mast Cell Disease Society.Medications to treat mast cell diseases.González-de-Olano D, Matito A, Orfao A, Escribano L.Advances in the understanding and clinical management of mastocytosis and clonal mast cell activation syndromes.F1000Res. 2016 Nov 14;5:2666. doi:10.12688/f1000research.9565.1Sciumè M, De Magistris C, Galli N, Ferretti E, Milesi G, De Roberto P, Fabris S, Grifoni FI.Target therapies for systemic mastocytosis: an update.Pharmaceuticals (Basel). 2022 Jun 11;15(6):738. doi:10.3390/ph15060738National Organization for Rare Disorders (NORD).Mastocytosis.ASCO Post (American Society of Clinical Oncology).FDA approves avapritinib for indolent systemic mastocytosis.Slapnicar C, Trinkaus M, Hicks L, Vadas P.Efficacy of omalizumab in indolent systemic mastocytosis.Case Rep Hematol. 2019 Sep 16;2019:3787586. doi:10.1155/2019/3787586The Institute for Functional Medicine.Fight inflammation and stabilize mast cells naturally.It’sSMthing: Systemic Mastocytosis.With many symptoms come many questions.National Comprehensive Cancer Network (NCCN).NCCN guidelines for patients: systemic mastocytosis.Mikkelsen CS, Nybo A, Arvesen KB, Holk-Poulsen J.Delayed diagnosis of adult indolent systemic mastocytosis.Dermatol Reports. 2014 Feb 17;6(1):5199. doi:10.4081/dr.2014.5199Gotlib J., Gerds, AT, Abdelmessieh, P, Ali, H, et al.NCCN guidelines insights: systemic mastocytosis, version 3.2024: featured updates to the NCCN guidelines.Journal of the National Comprehensive Cancer Network, 2024;22(2D)e240030. doi:10.6004/jnccn.2024.0030Nilsson A, Ungerstedt J, Hedström M. Living with systemic mastocytosis:Balancing between vulnerability and resilience: a qualitative study.European Journal of Oncology Nursing. October 2022;60;102172. doi:10.1016/j.ejon.2022.102172
Merck Manual Professional Version.Mastocytosis and mast cell activation syndrome.
Buonomo A, Nucera E, Criscuolo M.Treatment of indolent and advanced systemic mastocytosis.Mediterr J Hematol Infect Dis. 2022 May 1;14(1):e2022040. doi:10.4084/MJHID.2022
AB Science.Indolent systemic mastocytosis.
Pardanani A.How I treat patients with indolent and smoldering mastocytosis (rare conditions but difficult to manage).Blood. 2013;121(16)3085-3094. doi:10.1182/blood-2013-01-453183
UWHealth.Mast cell activation syndrome and diet.
NHS.Overview: mastocytosis.
Allergy and Asthma Network.What is anaphylaxis?
Czarny J, Lange M, Ługowska-Umer H, Nowicki RJ.Cutaneous mastocytosis treatment: strategies, limitations and perspectives.Postepy Dermatol Alergol. 2018 Dec;35(6):541-545. doi:10.5114/ada.2018.77605
The Mast Cell Disease Society.Medications to treat mast cell diseases.
González-de-Olano D, Matito A, Orfao A, Escribano L.Advances in the understanding and clinical management of mastocytosis and clonal mast cell activation syndromes.F1000Res. 2016 Nov 14;5:2666. doi:10.12688/f1000research.9565.1
Sciumè M, De Magistris C, Galli N, Ferretti E, Milesi G, De Roberto P, Fabris S, Grifoni FI.Target therapies for systemic mastocytosis: an update.Pharmaceuticals (Basel). 2022 Jun 11;15(6):738. doi:10.3390/ph15060738
National Organization for Rare Disorders (NORD).Mastocytosis.
ASCO Post (American Society of Clinical Oncology).FDA approves avapritinib for indolent systemic mastocytosis.
Slapnicar C, Trinkaus M, Hicks L, Vadas P.Efficacy of omalizumab in indolent systemic mastocytosis.Case Rep Hematol. 2019 Sep 16;2019:3787586. doi:10.1155/2019/3787586
The Institute for Functional Medicine.Fight inflammation and stabilize mast cells naturally.
It’sSMthing: Systemic Mastocytosis.With many symptoms come many questions.
National Comprehensive Cancer Network (NCCN).NCCN guidelines for patients: systemic mastocytosis.
Mikkelsen CS, Nybo A, Arvesen KB, Holk-Poulsen J.Delayed diagnosis of adult indolent systemic mastocytosis.Dermatol Reports. 2014 Feb 17;6(1):5199. doi:10.4081/dr.2014.5199
Gotlib J., Gerds, AT, Abdelmessieh, P, Ali, H, et al.NCCN guidelines insights: systemic mastocytosis, version 3.2024: featured updates to the NCCN guidelines.Journal of the National Comprehensive Cancer Network, 2024;22(2D)e240030. doi:10.6004/jnccn.2024.0030
Nilsson A, Ungerstedt J, Hedström M. Living with systemic mastocytosis:Balancing between vulnerability and resilience: a qualitative study.European Journal of Oncology Nursing. October 2022;60;102172. doi:10.1016/j.ejon.2022.102172
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