Urticaria pigmentosaUUrticaria pigmentosaUrticaria pigmentosaEnglishDermatologyChild (0-12 years);Teen (13-18 years)SkinSkinConditions and diseasesCaregivers Adult (19+)NA2016-06-24T04:00:00ZShanna Spring, MD, FRCPC, FAAD;Irene Lara-Corrales, MSc, MD​​​9.8000000000000048.4000000000000908.000000000000Health (A-Z) - ConditionsHealth A-Z<p>Urticaria pigmentosa is a harmless condition that produces red-brown spots or bumps on the skin. Learn how it is caused, diagnosed and treated.</p><h2>What is urticaria pigmentosa?</h2> <figure> <img src="https://assets.aboutkidshealth.ca/akhassets/PMD_urticaria_pigmentosa_EN.jpg" alt="The back of a baby with urticaria pigmentosa" /> </figure> <p>Our immune system contains different types of cells, including mast cells. These cells help the body heal after a wound and respond to possible allergens.</p><p>Sometimes mast cells build up in large numbers outside their usual environment, causing a condition called mastocytosis. Cutaneous mastocytosis occurs when mast cells build up in skin, hair or nails. The most common type of cutaneous mastocytosis in children is urticaria pigmentosa (UP). The condition is also called maculopapular mastocytosis.</p><p>Over half of all cases of UP first appear in children aged under two. New spots can continue to appear over the next few years.<br></p><p>Like most childhood cutaneous mastocytoses, urticaria pigmentosa is benign (harmless). It is limited to the skin and there is little risk of symptoms that involve other organs.</p><p>UP is not a permanent disease. About half of all cases resolve by puberty and the other half improve greatly by then.</p><h2>Key points</h2> <ul> <li>Urticaria pigmentosa is the most common type of childhood cutaneous mastocytosis.</li> <li>Most UP appears before the age of two and often disappears or improves greatly by puberty.</li> <li>In most children, symptoms are limited to redness, swelling and itchiness, but diarrhea, vomiting, flushing, wheezing and anaphylaxis can also occur.</li> <li>There is no cure for UP. Treatment is aimed at relieving symptoms by using medications and avoiding triggers.</li> <li>See a doctor for UP if your child shows signs of a severe allergic reaction. See a dermatologist if your child develops many new spots or has UP along with vomiting and diarrhea.</li> </ul><h2>How does urticaria pigmentosa affect the body?</h2> <figure> <span class="asset-image-title">Positive Darier’s sign</span> <img src="https://assets.aboutkidshealth.ca/akhassets/PMD_urticaria_pigmentosa_Dariers_sign_EN.jpg" alt="" /> </figure> <p>Urticaria pigmentosa most commonly appears as red-brown spots or bumps and can affect any part of the body. The number of spots varies from child to child, from just a few to hundreds.</p><p>These spots are commonly itchy. After vigorous rubbing, they can become red and swollen, indicating that the mast cells are activated. Your doctor might describe the redness and swelling as a positive Darier’s sign.<br></p><p>Besides being red and itchy, the spots associated with UP can sometimes become fluid-filled blisters. They usually do not leave a scar, but they can linger for months to years.</p><p>UP is limited to the skin in most children, but, in rare cases, it can produce other symptoms such as:</p><ul><li> <a href="https://akhpub.aboutkidshealth.ca/article?contentid=7&language=English">diarrhea</a><br></li><li> <a href="https://akhpub.aboutkidshealth.ca/article?contentid=347&language=English">vomiting</a></li><li>flushing</li><li>wheezing<br></li><li> <a href="https://akhpub.aboutkidshealth.ca/article?contentid=781&language=English">anaphylaxis</a> (serious allergic reactions).</li></ul><p>If your child has any of these unusual symptoms, tell your doctor at your next visit.<br></p><h2>What causes urticaria pigmentosa?</h2> <p>The cause of urticaria pigmentosa is unknown, but it is thought that genetics may play a part.</p><h2>How is urticaria pigmentosa diagnosed?</h2> <p>Urticaria pigmentosa is easily diagnosed in clinic if a child under age two has red-brown spots and a positive Darier’s sign. When the diagnosis is more uncertain, your child’s doctor may order a <a href="https://akhpub.aboutkidshealth.ca/article?contentid=2464&language=English">skin biopsy</a> to examine the spots.</p> <p>Once UP is diagnosed, a dermatologist will monitor your child every six to 12 months.</p> <p>Usually your child will not need further testing. However, your doctor may consider doing a blood test or may refer your child to a hematologist (a blood specialist) if:</p> <ul> <li>your child has many spots</li> <li>the spots look unusual</li> <li>any flare ups (redness and swelling) take longer to resolve than usual<br></li> <li>your child has severe systemic complaints, such as severe vomiting, diarrhea or wheezing.</li> </ul><h2>How is urticaria pigmentosa treated?</h2> <p>Unfortunately, there is no cure for UP. However, most children affected by the disease improve by puberty. In the meantime, the symptoms of UP can be treated successfully with a range of approaches.</p> <h3>Avoiding triggers</h3> <p>The website Mastokids features a <a href="http://www.mastokids.org/degranular-list" target="_blank">detailed list of possible triggers</a> and how to monitor your child for symptoms if you cannot avoid them.</p> <p>Examples of common triggers include:</p> <ul> <li>medications such as aspirin, NSAIDs (such as ibuprofen), narcotics, general anaesthetics (usually older agents that are no longer being used) and x-ray contrast dye<br></li> <li>stimuli such as stress, heat and <a href="https://akhpub.aboutkidshealth.ca/article?contentid=308&language=English">direct sunlight</a>.</li> </ul> <p>Always tell your child’s doctor if your child has UP so they can avoid using or recommending anything that may trigger your child’s symptoms.</p> <h3>Medications</h3> <ul> <li>Antihistamines can help relieve the itchiness and other side effects of mast cell activation, such as redness and swelling.</li> <li>Corticosteroid creams can provide itch relief.</li> <li>Cromolyn sodium (an anti-inflammatory medication given through an inhaler or by mouth) helps treat any diarrhea, vomiting or other gastrointestinal complaints.</li> <li>An <a href="https://akhpub.aboutkidshealth.ca/article?contentid=130&language=English">EpiPen</a> can help treat severe allergic reactions. Your child should carry one with them at all times if they have a history of <a href="https://akhpub.aboutkidshealth.ca/article?contentid=781&language=English">anaphylaxis</a>.</li> </ul> <h3>Light therapy</h3> <p>Light therapy (PUVA) has been used successfully in some severe cases.</p><h2>Complications of urticaria pigmentosa </h2> <p>In a very small number of cases, mast cells can build up in the liver, spleen and bone marrow. This is such a rare occurrence in children that regular screening is not recommended. Adults with UP can sometimes develop leukemia, but this is extremely rare in children.</p><h2>When to see a doctor for urticaria pigmentosa</h2> <ul> <li>Go to the emergency room if your child shows any signs of a severe allergic reaction or difficulty breathing.</li> <li>See your child’s dermatologist if your child develops many more or many different types of spots or if your child has new symptoms such as vomiting or diarrhea.</li> </ul>​ <h2>Further information</h2><p>For more information on urticaria pigmentosa, see <a target="_blank" href="https://www.mastokids.org/">Mastokids.org​</a>, a website to support children and families living with mastocytosis.​</p>
Urticaire pigmentaireUUrticaire pigmentaireUrticaria pigmentosaFrenchDermatologyChild (0-12 years);Teen (13-18 years)SkinSkinConditions and diseasesCaregivers Adult (19+)NA2016-04-26T04:00:00ZShanna Spring, MD, FRCPC, FAAD;Irene Lara-Corrales, MSc, MD​​​10.000000000000050.0000000000000863.000000000000Health (A-Z) - ConditionsHealth A-Z<p>L’urticaire pigmentaire est un trouble bénin de la peau. Découvrez ce qui la cause, comment elle est diagnostiquée et comment elle est soignée.</p><br><h2>Qu’est-ce que l’urticaire pigmentaire?</h2> <figure> <img src="https://assets.aboutkidshealth.ca/akhassets/PMD_urticaria_pigmentosa_FR.jpg" alt="Le dos d’un bébé atteint de l’urticaire pigmentaire" /> </figure> <p>Le système immunitaire est formé de différents types de cellules, dont les mastocytes. Ces cellules aident le corps à guérir après une blessure et à réagir aux allergènes.</p><p>Parfois, les mastocytes s’accumulent en grand nombre en-dehors de leur champ habituel. Cela cause un trouble appelé la mastocytose. On est en présence d’une mastocytose cutanée quand les mastocytes s’accumulent sur la peau, sur les cheveux ou sur les ongles. Le type le plus commun de mastocytose cutanée chez l’enfant est l’urticaire pigmentaire. Le trouble est aussi connu sous le nom de mastocytose maculo-papuleuse.</p><p>Dans plus de la moitié des cas, l’urticaire pigmentaire se manifeste d’abord chez les enfants âgés de moins de deux ans. De nouvelles taches peuvent apparaître au cours des prochaines années.</p><p>Comme la plupart des mastocytoses cutanées de l’enfance, l’urticaire pigmentaire est bénigne (sans danger). Elle se limite à la peau. Il y a peu de risque de symptômes qui mettent en cause d’autres organes.</p><p>Il ne s’agit pas d’un trouble permanent. Environ la moitié des cas disparaissent avant la puberté et l’autre moitié s’améliore grandement avant ce stage de croissance.<br></p><h2>À retenir</h2><ul><li>L’urticaire pigmentaire est le type le plus courant de mastocytose cutanée chez les enfants. </li><li>La plupart de temps, ce trouble se déclare avant l’âge de deux ans. Il disparaît ou s’améliore considérablement avant la puberté.</li><li>Chez la plupart des enfants, les symptômes se limitent aux rougeurs, aux enflures et aux démangeaisons. Mais, la diarrhée, les vomissements, les bouffées de chaleur, la respiration sifflante et l’anaphylaxie (réaction allergique grave) peuvent aussi se produire.</li><li>Il n’y a pas de remède pour l’urticaire pigmentaire. Le traitement vise à soulager les symptômes. Il comprend prendre des médicaments et éviter les éléments déclencheurs.</li><li>Voir un médecin pour l’urticaire pigmentaire si votre enfant présente des signes d’une réaction allergique grave. Voir un dermatologue si votre enfant a beaucoup de nouvelles taches ou si l’urticaire pigmentaire est accompagnée de vomissements et de diarrhée.<br></li></ul><h2>Quels sont les effets de l’urticaire pigmentaire sur le corps?</h2> <figure><span class="asset-image-title">Les signes de la maladie de Darier</span><img src="https://assets.aboutkidshealth.ca/akhassets/PMD_urticaria_pigmentosa_Dariers_sign_EN.jpg" alt="" /> </figure> <p>L’urticaire pigmentaire se manifeste le plus souvent par des taches ou des bosses rouge brunâtre. Elle peut toucher n’importe quelle partie du corps. Le nombre de taches varie d’un enfant à l’autre, de quelques-unes à des centaines. </p><p>Ces taches sont souvent accompagnées de démangeaisons. Après avoir été frottées vigoureusement, elles peuvent devenir rouges et enflées. Il s’agit d’une indication que les mastocytes sont activées. Il se peut que votre médecin décrive la rougeur et l’enflure comme des signes de la maladie de Darier.</p><p>En plus d’être rouges et de causer des démangeaisons, les taches se transforment parfois en cloques remplies de fluide Elles ne laissent normalement pas de cicatrice, mais elles peuvent persister pendant des mois, voire même des années.</p><p>Chez la plupart des enfants, ce trouble n’a des effets que sur la peau. Dans de rares cas, l’enfant peut présenter d’autres symptômes. En voici quelques-uns :<br></p><ul><li>la <a href="https://akhpub.aboutkidshealth.ca/article?contentid=7&language=French">diarrhée</a></li><li>des <a href="https://akhpub.aboutkidshealth.ca/article?contentid=347&language=French">vomissements</a></li><li>des bouffées de chaleur</li><li>une respiration sifflante</li><li>une <a href="https://akhpub.aboutkidshealth.ca/article?contentid=781&language=French">anaphylaxie</a> (réaction allergique grave) (en anglais).</li></ul><p>Si votre enfant présente un de ces symptômes anormaux, prévenez votre médecin lors de votre prochaine visite.<br></p><h2>Quelle est la cause de l’urticaire pigmentaire?</h2><p>La cause de l’urticaire pigmentaire n’est pas connue. On pense que la génétique peut jouer un rôle.</p><h2>Comment l’urticaire pigmentaire est-elle diagnostiquée?</h2><p>Il est facile de porter un diagnostic d’urticaire pigmentaire en clinique si un enfant âgé de moins de deux ans a des taches rouge brunâtre et présente un signe de la maladie de Darier. Quand le diagnostic n’est pas arrêté, le médecin de votre enfant peut demander une <a href="https://akhpub.aboutkidshealth.ca/article?contentid=2464&language=French">biopsie cutanée</a> afin de faire analyser les taches.<br></p><p>Une fois que le diagnostic est porté, un dermatologue verra votre enfant tous les six à 12 mois.</p><p>En général, votre enfant n’aura pas besoin de nouveaux examens. Votre médecin peut décider de faire des analyses sanguines ou d'adresser votre enfant à un hématologue (spécialiste du sang) lorsqu’il présente l’un des symptômes suivants :</p><ul><li>sa peau est couverte de nombreuses taches;</li><li>ses taches paraissent anormales;</li><li>ses poussées actives (rougeur et enflure) prennent plus de temps à disparaître que d’habitude;</li><li>il a des problèmes systémiques graves, tels des vomissements violents, une diarrhée aiguë ou une respiration sifflante.<br></li></ul><h2>Comment l’urticaire pigmentaire est-elle soignée?</h2><p>Malheureusement, il n’y a pas de remède pour l’urticaire pigmentaire. Cependant, la plupart des enfants atteints voient leur état s’améliorer avant la puberté. En attendant, les symptômes peuvent être soignés grâce à un éventail de méthodes de traitement.<br></p><h3>Éviter les éléments déclencheurs</h3><p>Le site web Mastokids présente une <a href="http://www.mastokids.org/degranular-list" target="_blank">liste détaillée des éléments déclencheurs possibles</a> (en anglais seulement) et comment déceler les symptômes chez votre enfant si vous ne pouvez pas les éviter.</p><p>Exemples d’éléments déclencheurs courants :</p><ul><li>les médicaments comme l’aspirine, l’AINS (comme l’ibuprofène), les narcotiques, les anesthésiques généraux (souvent des agents plus anciens qui ne sont plus utilisés) et la teinture radiographique;</li><li>des stimuli, tels le stress, la chaleur et l’exposition <a href="https://akhpub.aboutkidshealth.ca/article?contentid=308&language=French">direc​te au soleil</a>.</li></ul><p>Dites toujours au médecin si votre enfant souffre d’urticaire pigmentaire afin d'éviter l'utilisation ou la recommandation de tout élément qui pourrait déclencher chez lui des symptômes.</p><h3>Médicaments</h3><ul><li>Les antihistaminiques peuvent atténuer les démangeaisons et les autres effets secondaires de l’activation des mastocytes, comme la rougeur et l’enflure.</li><li>Les crèmes de corticostéroïdes peuvent atténuer les démangeaisons.</li><li>Le cromoglycate disodique (un médicament anti-inflammatoire donné par inhalateur ou par voie orale) permet de soigner la diarrhée, des vomissements ou autres problèmes gastro-intestinaux.</li><li>Un <a href="https://akhpub.aboutkidshealth.ca/article?contentid=130&language=French">EpiPen</a>​ peut aider à soigner les réactions allergiques graves. Votre enfant devrait avoir ce médicament avec lui en permanence s’il a des antécédents d’<a href="https://akhpub.aboutkidshealth.ca/article?contentid=781&language=French">anaphylaxie</a>.</li></ul><h3>Photothérapie</h3><p>La photothérapie (PUVA) a été utilisée avec succès dans certains cas graves.</p><h2>Complications de l’urticaire pigmentaire</h2><p>Dans un très petit nombre de cas, les mastocytes peuvent s’accumuler dans le foie, la rate et la moelle osseuse. Cela est si rare chez l’enfant que le dépistage régulier n’est pas recommandé. Les adultes atteints d’urticaire pigmentaire sont parfois à risque d’une leucémie, mais cela est très rare chez les enfants.<br></p><h2>Quand consulter un médecin pour l’urticaire pigmentaire</h2><ul><li>Se présenter au service d’urgence si votre enfant présente des signes d’une réaction allergique grave ou s’il a de la difficulté à respirer.</li><li>Voir le dermatologue si votre enfant a beaucoup plus de taches ou de différentes sortes de taches. Aussi, s’il présente de nouveaux symptômes, comme des vomissements ou la diarrhée.<br></li></ul>

 

 

 

 

Urticaria pigmentosa2301.00000000000Urticaria pigmentosaUrticaria pigmentosaUEnglishDermatologyChild (0-12 years);Teen (13-18 years)SkinSkinConditions and diseasesCaregivers Adult (19+)NA2016-06-24T04:00:00ZShanna Spring, MD, FRCPC, FAAD;Irene Lara-Corrales, MSc, MD​​​9.8000000000000048.4000000000000908.000000000000Health (A-Z) - ConditionsHealth A-Z<p>Urticaria pigmentosa is a harmless condition that produces red-brown spots or bumps on the skin. Learn how it is caused, diagnosed and treated.</p><h2>What is urticaria pigmentosa?</h2> <figure> <img src="https://assets.aboutkidshealth.ca/akhassets/PMD_urticaria_pigmentosa_EN.jpg" alt="The back of a baby with urticaria pigmentosa" /> </figure> <p>Our immune system contains different types of cells, including mast cells. These cells help the body heal after a wound and respond to possible allergens.</p><p>Sometimes mast cells build up in large numbers outside their usual environment, causing a condition called mastocytosis. Cutaneous mastocytosis occurs when mast cells build up in skin, hair or nails. The most common type of cutaneous mastocytosis in children is urticaria pigmentosa (UP). The condition is also called maculopapular mastocytosis.</p><p>Over half of all cases of UP first appear in children aged under two. New spots can continue to appear over the next few years.<br></p><p>Like most childhood cutaneous mastocytoses, urticaria pigmentosa is benign (harmless). It is limited to the skin and there is little risk of symptoms that involve other organs.</p><p>UP is not a permanent disease. About half of all cases resolve by puberty and the other half improve greatly by then.</p><h2>Key points</h2> <ul> <li>Urticaria pigmentosa is the most common type of childhood cutaneous mastocytosis.</li> <li>Most UP appears before the age of two and often disappears or improves greatly by puberty.</li> <li>In most children, symptoms are limited to redness, swelling and itchiness, but diarrhea, vomiting, flushing, wheezing and anaphylaxis can also occur.</li> <li>There is no cure for UP. Treatment is aimed at relieving symptoms by using medications and avoiding triggers.</li> <li>See a doctor for UP if your child shows signs of a severe allergic reaction. See a dermatologist if your child develops many new spots or has UP along with vomiting and diarrhea.</li> </ul><h2>How does urticaria pigmentosa affect the body?</h2> <figure> <span class="asset-image-title">Positive Darier’s sign</span> <img src="https://assets.aboutkidshealth.ca/akhassets/PMD_urticaria_pigmentosa_Dariers_sign_EN.jpg" alt="" /> </figure> <p>Urticaria pigmentosa most commonly appears as red-brown spots or bumps and can affect any part of the body. The number of spots varies from child to child, from just a few to hundreds.</p><p>These spots are commonly itchy. After vigorous rubbing, they can become red and swollen, indicating that the mast cells are activated. Your doctor might describe the redness and swelling as a positive Darier’s sign.<br></p><p>Besides being red and itchy, the spots associated with UP can sometimes become fluid-filled blisters. They usually do not leave a scar, but they can linger for months to years.</p><p>UP is limited to the skin in most children, but, in rare cases, it can produce other symptoms such as:</p><ul><li> <a href="https://akhpub.aboutkidshealth.ca/article?contentid=7&language=English">diarrhea</a><br></li><li> <a href="https://akhpub.aboutkidshealth.ca/article?contentid=347&language=English">vomiting</a></li><li>flushing</li><li>wheezing<br></li><li> <a href="https://akhpub.aboutkidshealth.ca/article?contentid=781&language=English">anaphylaxis</a> (serious allergic reactions).</li></ul><p>If your child has any of these unusual symptoms, tell your doctor at your next visit.<br></p><h2>What causes urticaria pigmentosa?</h2> <p>The cause of urticaria pigmentosa is unknown, but it is thought that genetics may play a part.</p><h2>How is urticaria pigmentosa diagnosed?</h2> <p>Urticaria pigmentosa is easily diagnosed in clinic if a child under age two has red-brown spots and a positive Darier’s sign. When the diagnosis is more uncertain, your child’s doctor may order a <a href="https://akhpub.aboutkidshealth.ca/article?contentid=2464&language=English">skin biopsy</a> to examine the spots.</p> <p>Once UP is diagnosed, a dermatologist will monitor your child every six to 12 months.</p> <p>Usually your child will not need further testing. However, your doctor may consider doing a blood test or may refer your child to a hematologist (a blood specialist) if:</p> <ul> <li>your child has many spots</li> <li>the spots look unusual</li> <li>any flare ups (redness and swelling) take longer to resolve than usual<br></li> <li>your child has severe systemic complaints, such as severe vomiting, diarrhea or wheezing.</li> </ul><h2>How is urticaria pigmentosa treated?</h2> <p>Unfortunately, there is no cure for UP. However, most children affected by the disease improve by puberty. In the meantime, the symptoms of UP can be treated successfully with a range of approaches.</p> <h3>Avoiding triggers</h3> <p>The website Mastokids features a <a href="http://www.mastokids.org/degranular-list" target="_blank">detailed list of possible triggers</a> and how to monitor your child for symptoms if you cannot avoid them.</p> <p>Examples of common triggers include:</p> <ul> <li>medications such as aspirin, NSAIDs (such as ibuprofen), narcotics, general anaesthetics (usually older agents that are no longer being used) and x-ray contrast dye<br></li> <li>stimuli such as stress, heat and <a href="https://akhpub.aboutkidshealth.ca/article?contentid=308&language=English">direct sunlight</a>.</li> </ul> <p>Always tell your child’s doctor if your child has UP so they can avoid using or recommending anything that may trigger your child’s symptoms.</p> <h3>Medications</h3> <ul> <li>Antihistamines can help relieve the itchiness and other side effects of mast cell activation, such as redness and swelling.</li> <li>Corticosteroid creams can provide itch relief.</li> <li>Cromolyn sodium (an anti-inflammatory medication given through an inhaler or by mouth) helps treat any diarrhea, vomiting or other gastrointestinal complaints.</li> <li>An <a href="https://akhpub.aboutkidshealth.ca/article?contentid=130&language=English">EpiPen</a> can help treat severe allergic reactions. Your child should carry one with them at all times if they have a history of <a href="https://akhpub.aboutkidshealth.ca/article?contentid=781&language=English">anaphylaxis</a>.</li> </ul> <h3>Light therapy</h3> <p>Light therapy (PUVA) has been used successfully in some severe cases.</p><h2>Complications of urticaria pigmentosa </h2> <p>In a very small number of cases, mast cells can build up in the liver, spleen and bone marrow. This is such a rare occurrence in children that regular screening is not recommended. Adults with UP can sometimes develop leukemia, but this is extremely rare in children.</p><h2>When to see a doctor for urticaria pigmentosa</h2> <ul> <li>Go to the emergency room if your child shows any signs of a severe allergic reaction or difficulty breathing.</li> <li>See your child’s dermatologist if your child develops many more or many different types of spots or if your child has new symptoms such as vomiting or diarrhea.</li> </ul>​ <h2>Further information</h2><p>For more information on urticaria pigmentosa, see <a target="_blank" href="https://www.mastokids.org/">Mastokids.org​</a>, a website to support children and families living with mastocytosis.​</p>https://assets.aboutkidshealth.ca/akhassets/PMD_urticaria_pigmentosa_Dariers_sign_EN.jpgUrticaria pigmentosaFalse