Table of ContentsView AllTable of ContentsSkin EffectsWho Gets It?Link to MastocytosisHealthcare Providers to SeeAt-Home TreatmentPrescription TreatmentManaging TriggersComplicatonsManaging a Chronic Form
Table of ContentsView All
View All
Table of Contents
Skin Effects
Who Gets It?
Link to Mastocytosis
Healthcare Providers to See
At-Home Treatment
Prescription Treatment
Managing Triggers
Complicatons
Managing a Chronic Form
Urticaria pigmentosa, also called maculopapular cutaneous mastocytosis, is a skin condition that causes spots on the skin and skin lesions.It stems from having too many mast cells in the skin, a type of white blood cell involved in the immune system.
Understanding how these symptoms manifest and their connection to mastocytosis can help identify the condition and seek appropriate treatment.
This photo contains content that some people may find graphic or disturbing.See PhotoReproduced with permission from © DermNetdermnetnz.org2023.
This photo contains content that some people may find graphic or disturbing.See Photo
This photo contains content that some people may find graphic or disturbing.
Reproduced with permission from © DermNetdermnetnz.org2023.
Urticaria Pigmentosa and Skin Effects
Urticaria pigmentosa is a skin condition often seen in systemic mastocytosis, in which too many mast cells in the skin cause itchy, brownish, patches of skin that can develop into hive-like bumps. Children may develop fluid-filled blisters.In darker skin tones, they may look purple or violet-colored.
Affected Body Parts
Urticaria pigmentosa can appear on any part of the body. It commonly affects the following areas:
When these skin lesions begin in infancy or during childhood, the skin is usually the only affected organ.
Textures and Sensations
The lesions in urticaria pigmentosa appear as itchy, brownish patches of skin that may form hives when scratched. There be only a few spots or many spots covering a large area.
Rubbing an urticaria pigmentosa lesion can make the area turn red, swollen, and itchy. A blister may occur, a reaction known as Darier’s sign, which helps diagnose the condition. In young children, these patches often blister when rubbed. If many patches are activated at once, the child might become itchy and irritable, though severe symptoms are rare.
Sometimes, mastocytosis in the skin can cause a massive histamine (chemical that can cause an allergic reaction) release. If this occurs, there may be additional symptoms, including:
Symptom Onset and Duration
Many infants and children tend to outgrow urticaria pigmentosa over time. No treatment is currently known to prevent their formation. The reddish-brown spots often diminish as the child ages but typically persist for several years.
Who Gets Urticaria Pigmentosa?
Urticaria pigmentosa happens when the body’s mast cells release histamines in response to a perceived threat to the immune system. These histamines cause the characteristic swelling and itching of the skin in urticaria pigmentosa. Anything that triggers the release of histamines can cause someone to get urticaria pigmentosa.
Certain factors can cause urticaria pigmentosa, including:
Link Between Urticaria Pigmentosa and Mastocytosis
Mastocytosis itself can be:
Systemic mastocytosis is a more serious form that affects multiple organs. While urticaria pigmentosa mostly affects the skin, in some cases, it can lead to systemic (body-wide) symptoms if mast cells spread to other parts of the body, like the bone marrow, brain, nerves, or digestive system. This progression means healthcare providers must watch for signs of the condition spreading beyond the skin.
Understanding this link helps healthcare providers monitor and treat urticaria pigmentosa and related mastocytosis.
Who to See for Urticaria Pigmentosa Diagnosis
Diagnosing urticaria pigmentosa typically involves consulting with specialists such as:
How to Soothe Urticaria Pigmentosa at Home
Before trying home remedies, talk to your healthcare provider to ensure you use an effective and safe treatment. Home remedies that may help manage symptoms and provide relief include:
Prescribed Urticaria Pigmentosa Treatment
Prescription medications play a crucial role in managing urticaria pigmentosa symptoms.
Oral antihistaminesare commonly prescribed to help alleviate itching and reduce the release of histamine from mast cells.
Mast cell stabilizers(like cromolyn sodium) or leukotriene inhibitors (such as montelukast) may be used to prevent mast cell activation and the subsequent release of inflammatory mediators.
Topical or oral corticosteroidsare another treatment option for localized urticaria pigmentosa lesions that are particularly bothersome or resistant to other treatments. These medications help reduce inflammation and improve skin symptoms.
In cases where lesions are extensive or cause significant discomfort, phototherapy (light therapy) using ultraviolet A (UVA) or narrowband ultraviolet B (UVB) light may be recommended to help suppress mast cell activity in the skin.
Ttreatment for urticaria pigmentosa depends on the severity of symptoms and the patient’s response to various therapies. Regular follow-up with a dermatologist or allergist-immunologist is essential to monitor the condition, adjust treatment as needed, and address any systemic symptoms that may develop over time.
Managing and Staying Ahead of Triggers
Triggers for urticaria pigmentosa are factors that can cause mast cells in the skin to release their contents, leading to symptoms such as itching, swelling, and redness of skin lesions. Common triggers include:
Identifying and avoiding these triggers can help individuals with urticaria pigmentosa manage their symptoms more effectively and reduce the frequency of flare-ups.
Secondary Urticaria Pigmentosa Complications
Secondary complications of urticaria pigmentosa can arise due to the chronic inflammatory and allergic nature of the condition, particularly when mast cells accumulate and become activated in the skin. Some of these complications include:
Systemic Symptoms
While urticaria pigmentosa primarily affects the skin, some individuals may develop systemic mastocytosis, where mast cells accumulate in other organs such as the bone marrow, liver, or gastrointestinal tract. In very rare cases, it can turn into:
Anaphylaxis
In severe cases, widespread activation of mast cells in response to triggers like insect stings, certain medications, or foods can lead to a systemic allergic reaction known as anaphylaxis.Anaphylaxis is a medical emergency requiring immediate treatment with epinephrine and urgent medical care.
Chronic Urticaria Pigmentosa Management
Living with urticaria pigmentosa involves regular check-ups with healthcare providers to monitor the condition and adjust treatments as needed. It may also help to connect with others via online support groups for support and advice.
Summary
13 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.DermNet.Maculopapular cutaneous mastocytosis.MedlinePlus.Urticaria pigmentosa.DermNet.Mastocytosis.Children’s Health.Urticaria pigmentosa.National Organization for Rare Disorders.Mastocytosis.Children’s Mercy.Urticaria pigmentosa (mastocytosis of the skin).Immune deficiency foundation.Mast cell aestivation disease diagnosis can reveal PI.American Society of Hematology.How I treat patients with advanced systemic mastocytosis.American Academy or Dermatology Association.How to relieve itchy skin.My Eczema Team.Oatmeal bath for eczema: effectiveness, benefits, and uses.Mast Cell Hope.Treatment of mast cell disease.The Mast Cell Disease Society.Symptoms and triggers of mast cell activation.MedlinePlus.Anaphylaxis.
13 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.DermNet.Maculopapular cutaneous mastocytosis.MedlinePlus.Urticaria pigmentosa.DermNet.Mastocytosis.Children’s Health.Urticaria pigmentosa.National Organization for Rare Disorders.Mastocytosis.Children’s Mercy.Urticaria pigmentosa (mastocytosis of the skin).Immune deficiency foundation.Mast cell aestivation disease diagnosis can reveal PI.American Society of Hematology.How I treat patients with advanced systemic mastocytosis.American Academy or Dermatology Association.How to relieve itchy skin.My Eczema Team.Oatmeal bath for eczema: effectiveness, benefits, and uses.Mast Cell Hope.Treatment of mast cell disease.The Mast Cell Disease Society.Symptoms and triggers of mast cell activation.MedlinePlus.Anaphylaxis.
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.
DermNet.Maculopapular cutaneous mastocytosis.MedlinePlus.Urticaria pigmentosa.DermNet.Mastocytosis.Children’s Health.Urticaria pigmentosa.National Organization for Rare Disorders.Mastocytosis.Children’s Mercy.Urticaria pigmentosa (mastocytosis of the skin).Immune deficiency foundation.Mast cell aestivation disease diagnosis can reveal PI.American Society of Hematology.How I treat patients with advanced systemic mastocytosis.American Academy or Dermatology Association.How to relieve itchy skin.My Eczema Team.Oatmeal bath for eczema: effectiveness, benefits, and uses.Mast Cell Hope.Treatment of mast cell disease.The Mast Cell Disease Society.Symptoms and triggers of mast cell activation.MedlinePlus.Anaphylaxis.
DermNet.Maculopapular cutaneous mastocytosis.
MedlinePlus.Urticaria pigmentosa.
DermNet.Mastocytosis.
Children’s Health.Urticaria pigmentosa.
National Organization for Rare Disorders.Mastocytosis.
Children’s Mercy.Urticaria pigmentosa (mastocytosis of the skin).
Immune deficiency foundation.Mast cell aestivation disease diagnosis can reveal PI.
American Society of Hematology.How I treat patients with advanced systemic mastocytosis.
American Academy or Dermatology Association.How to relieve itchy skin.
My Eczema Team.Oatmeal bath for eczema: effectiveness, benefits, and uses.
Mast Cell Hope.Treatment of mast cell disease.
The Mast Cell Disease Society.Symptoms and triggers of mast cell activation.
MedlinePlus.Anaphylaxis.
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