Urticaria Pigmentosa

I. Definition: Urticaria pigmentosa is an uncommon rash that usually affects the neck, arms, legs and trunk of children and young adults. The rash consists of reddish-brown spots that turn into hives when they are rubbed hard or scratched. Sometimes the spots will blister.

II. Causes:

The spots in urticaria pigmentosa contain a large number of mast cells (cells are the bodies building blocks). Mast cells are immune cells (cells that fight infection) that live in the skin. Mast cells make a substance called histamine. Histamine causes hives, itching, and flushing. We do not known why people with urticaria pigmentosa have abnormal collections of mast cells in the skin.

III. Prognosis:

Most children who develop urticaria pigmentosa before the age of five will have resolution of the condition by adolescence or early adulthood. When urticaria pigmentosa starts after the age of five, the abnormal collections of mast cells will sometimes involve the internal organs and the disease does not always go away. These patients need blood tests and other studies.

IV. Treatment:

There is no satisfactory treatment for urticaria pigmentosa. Patients with urticaria pigmentosa should avoid aspirin, codeine, opiates, procaine, alcohol. polymyxin B, hot baths, and vigorous rubbing after bathing and showering. These can release histamine which can cause itching, flushing and hives.

Antihistamines such as Benadryl can help control itching, hives, and flushing.

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Next Result Set Image urticaria_pigmentosa_1_051204 File Type: jpg Name: URTICARIA PIGMENTOSA / cutaneous sign of Diagnosis: URTICARIA PIGMENTOSA, Category: systemic disease / DIFFUSE INFILTRATIVE hyperpigmentation total body / chest Body Site: Age: 5 years abdomen Contributor: Bernard Cohen, MD diffuse confluent hyperpigmented plaques with some overlying Description: urticarial plaques This generally healthy 5-year-old boy with diffuse urticaria pigmentosa complained of increasing abdominal pain. He had no Comments: diarrhea, no headaches, but intermittent pruritus. Endocsopy was scheduled to evaluate the gastrointestinal tract for mast cell involvement. All related Images urticaria_pigmentosa_4_051204 Related urticaria_pigmentosa_3_051204 Images: urticaria_pigmentosa_2_051204

2001-05, DermAtlas

urticaria_pigmentosa_1_051204 Image urticaria_pigmentosa_2_051204 File Type: jpg Name: URTICARIA PIGMENTOSA / cutaneous sign of Diagnosis: URTICARIA PIGMENTOSA, Category: systemic disease / DIFFUSE INFILTRATIVE hyperpigmentation total body / chest Body Site: Age: 5 years abdomen / face Contributor: Bernard Cohen, MD diffuse confluent hyperpigmented plaques with some overlying Description: urticarial plaques This generally healthy 5-year-old boy with diffuse urticaria pigmentosa complained of increasing abdominal pain. He had no Comments: diarrhea, no headaches, but intermittent pruritus. Endocsopy was scheduled to evaluate the gastrointestinal tract for mast cell involvement. All related Images urticaria_pigmentosa_4_051204 Related urticaria_pigmentosa_3_051204 Images: urticaria_pigmentosa_1_051204 Image urticaria_pigmentosa_3_051204 File Type: jpg Name: URTICARIA PIGMENTOSA / cutaneous sign of Diagnosis: URTICARIA PIGMENTOSA, Category: systemic disease / DIFFUSE INFILTRATIVE hyperpigmentation Body Site: total body / back Age: 5 years Contributor: Bernard Cohen, MD diffuse confluent hyperpigmented plaques with some overlying Description: urticarial plaques This generally healthy 5-year-old boy with diffuse urticaria pigmentosa complained of increasing abdominal pain. He had no Comments: diarrhea, no headaches, but intermittent pruritus. Endocsopy was scheduled to evaluate the gastrointestinal tract for mast cell involvement. All related Images urticaria_pigmentosa_4_051204 Related urticaria_pigmentosa_2_051204 Images: urticaria_pigmentosa_1_051204 Image urticaria_pigmentosa_4_051204 File Type: jpg Name: URTICARIA PIGMENTOSA / cutaneous sign of Diagnosis: URTICARIA PIGMENTOSA, Category: systemic disease / DIFFUSE INFILTRATIVE hyperpigmentation Body Site: total body / back Age: 5 years Contributor: Bernard Cohen, MD Description: diffuse confluent hyperpigmented plaques with some overlying

2001-05, DermAtlas

2001-05, DermAtlas

2001-05, DermAtlas

urticarial plaques This generally healthy 5-year-old boy with diffuse urticaria pigmentosa complained of increasing abdominal pain. He had no Comments: diarrhea, no headaches, but intermittent pruritus. Endocsopy was scheduled to evaluate the gastrointestinal tract for mast cell involvement. All related Images urticaria_pigmentosa_3_051204 Related urticaria_pigmentosa_2_051204 Images: urticaria_pigmentosa_1_051204 Image urticaria_pigmentosa_1_050523 Name: URTICARIA PIGMENTOSA / Diagnosis: HYPERPIGMENTATION File Type: jpg lumps & bumps (plaques, nodules, Category: tumors) / hyperpigmentation Age: 32 years

2001-05, DermAtlas

Body Site: foot Contributor: Bernard Cohen, MD multiple acral symmetric 2-3 mm golden brown macules and Description: slightly elevated papules This healthy 32-year-old woman had a 3 year history of brown macules and papules disseminated on her arms and legs with Comments: sparing of her trunk and face. A skin biopsy revealed an increased number of mast cells around dermal blood vessels. Related All related Images urticaria_pigmentosa_3_050523 Images: urticaria_pigmentosa_2_050523 Image urticaria_pigmentosa_2_050523 Name: Diagnosis: URTICARIA PIGMENTOSA / HYPERPIGMENTATION File Type: jpg

2001-05, DermAtlas

Body Site: foot Contributor: Bernard Cohen, MD multiple acral symmetric 2-3 mm golden brown macules and Description: slightly elevated papules This healthy 32-year-old woman had a 3 year history of brown macules and papules disseminated on her arms and legs with Comments: sparing of her trunk and face. A skin biopsy revealed an increased number of mast cells around dermal blood vessels. Related All related Images urticaria_pigmentosa_3_050523 Images: urticaria_pigmentosa_1_050523 Image urticaria_pigmentosa_3_050523 Name: Diagnosis: URTICARIA PIGMENTOSA / File Type: jpg Category: lumps & bumps

lumps & bumps (plaques, nodules, Category: tumors) / hyperpigmentation Age: 32 years

HYPERPIGMENTATION

2001-05, DermAtlas

Body Site: foot Contributor: Bernard Cohen, MD multiple acral symmetric 2-3 mm golden brown macules and Description: slightly elevated papules This healthy 32-year-old woman had a 3 year history of brown macules and papules disseminated on her arms and legs with Comments: sparing of her trunk and face. A skin biopsy revealed an increased number of mast cells around dermal blood vessels. Related All related Images urticaria_pigmentosa_2_050523 Images: urticaria_pigmentosa_1_050523 Image urticaria_pigmentosa_2_050114 Name: File Type: jpg

(plaques, nodules, tumors) / hyperpigmentation Age: 32 years

Diagnosis: URTICARIA PIGMENTOSA

2001-05, DermAtlas

Body Site: chest Contributor: Paul Honig, MD Description: bronze 1-2 cm round to oval leathery plaques with fuzzy borders Comments: This 16-month-old boy had a brown papule on his left thigh at birth. In the early months of his life, the patient developed new brown and tan papules on his chest, back, and occipital scalp. Although his growth and development were normal, some of the papules occasionally blistered after rubbing or irritation and he had a few brief episodes of flushing. Urticaria pigmentosa is one manifestation of cutaneous mastocytosis. This condition is characterized by persistent pruritic pigmented skin lesions that uriticate with mechanical or chemical irritation. This finding is known as Dariers sign. The condition is associated with mutations in the c-KIT protooncogene, which codes for the stem cell growth factor receptor (also known as the mast cell growth factor receptor). Urticaria pigmentosa can occur from birth to middle age with most cases occurring within the first 6 months of life. Cutaneous lesions consist of macules, papules, nodules, plaques, vesicle or bullae. Pruritus may be severe, and occurs as a result of the massive release of histamine from mast cell degranulation. Triggers for degranulation include alcohol, opiates, aspirin, quinine, scopolamine, gallamine, reserpine, amphotericin B, polymyxin, and tubocarine. On histology, a

lumps & bumps (plaques, nodules, tumors) / Category: hyperpigmentation / cutaneous sign of systemic disease Age: 16 months

dense dermal collection of mast cells is diagnostic of mastocytosis. Severe systemic disease may involve the lymph nodes, GI tract, bones, heart, blood, liver and spleen. The prognosis for isolated cutaneous involvement, such as in urticaria pigmentosa, is generally good. Most cases clear spontaenously or improve significantly over time. Symptomatic relief can be obtained with systemic anti-histamines. PUVA is frequently efficacious. Diarrhea as a result of urticaria pigmentosa can be controlled with oral cromolyn sodium. Avoidance of physical and chemical percipitants is of paramount importance for control of this condition. References: 1. Azana JM et al. Urticaria pigmentosa. Pediatr Dermatol. 1994;11:102. 2. Godt, O, et al. Short- and long-term effectiveness of oral and bath PUVA therapy in urticaria pigmentosa and systemic mastocytosis. Dermatology 1997, 195: 35. 3. Henz BM et al. SCF and c-kit in mastocytosis. J Invest Derm. 1998;110:186. 4. Topar G et al. Urticaria pigmentosa. Am J Clin Pathology 1998;109:279. 5. Longley J et al. The mast cell and mast cell disease. J Am Acad Dermatol 1995;32:545. 6. Tharp MD. Mast cell disease and its diagnosis. J Invest Derm. 1995;104:885. 7. Tharp, MD. Understanding mast cells and mastocytosis. J Invest Derm 1997, 108:698. Related urticaria_pigmentosa_1_050114 Images: Image urticaria_pigmentosa_1_050114 Name: File Type: jpg lumps & bumps (plaques, nodules, tumors) / Category: hyperpigmentation / cutaneous sign of systemic disease Age: 16 months

Diagnosis:

URTICARIA PIGMENTOSA / DARIER SIGN

2001-05, DermAtlas

Body Site: trunk / back Contributor: Paul Honig, MD bronze 1-2 cm round to oval leathery plaques with fuzzy borders Description: and one plaque with surrounding wheal Comments: This 16-month-old boy had a brown papule on his left thigh at birth. In the early months of his life, the patient developed new brown and tan papules on his chest, back, and occipital scalp. Although his growth and development were normal, some of the papules occasionally blistered after rubbing or irritation and he had a few brief episodes of flushing. Urticaria pigmentosa is one manifestation of cutaneous mastocytosis. This condition is characterized by persistent pruritic pigmented skin lesions that

uriticate with mechanical or chemical irritation. This finding is known as Dariers sign. The condition is associated with mutations in the c-KIT protooncogene, which codes for the stem cell growth factor receptor (also known as the mast cell growth factor receptor). Urticaria pigmentosa can occur from birth to middle age with most cases occurring within the first 6 months of life. Cutaneous lesions consist of macules, papules, nodules, plaques, vesicle or bullae. Pruritus may be severe, and occurs as a result of the massive release of histamine from mast cell degranulation. Triggers for degranulation include alcohol, opiates, aspirin, quinine, scopolamine, gallamine, reserpine, amphotericin B, polymyxin, and tubocarine. On histology, a dense dermal collection of mast cells is diagnostic of mastocytosis. Severe systemic disease may involve the lymph nodes, GI tract, bones, heart, blood, liver and spleen. The prognosis for isolated cutaneous involvement, such as in urticaria pigmentosa, is generally good. Most cases clear spontaenously or improve significantly over time. Symptomatic relief can be obtained with systemic anti-histamines. PUVA is frequently efficacious. Diarrhea as a result of urticaria pigmentosa can be controlled with oral cromolyn sodium. Avoidance of physical and chemical percipitants is of paramount importance for control of this condition. References: 1. Azana JM et al. Urticaria pigmentosa. Pediatr Dermatol. 1994;11:102. 2. Godt, O, et al. Short- and long-term effectiveness of oral and bath PUVA therapy in urticaria pigmentosa and systemic mastocytosis. Dermatology 1997, 195: 35. 3. Henz BM et al. SCF and c-kit in mastocytosis. J Invest Derm. 1998;110:186. 4. Topar G et al. Urticaria pigmentosa. Am J Clin Pathology 1998;109:279. 5. Longley J et al. The mast cell and mast cell disease. J Am Acad Dermatol 1995;32:545. 6. Tharp MD. Mast cell disease and its diagnosis. J Invest Derm. 1995;104:885. 7. Tharp, MD. Understanding mast cells and mastocytosis. J Invest Derm 1997, 108:698. Related urticaria_pigmentosa_2_050114 Images: Image urticaria_pigmentosa_1_040524 Name: Diagnosis:
2001-05, DermAtlas

File Type: jpg lumps & bumps Category: (plaques, nodules, tumors)

URTICARIA PIGMENTOSA / MASTOCYTOSIS

total body / forehead Age: 4 months shoulder Contributor: Mark Simonian, MD Description: multiple golden brown leathery papules Body Site:

This healthy 4-month-old boy developed asymptomatic scattered golden brown leathery papules on the scalp, shoulder, ankle, Comments: and leg. The presence of a Darier sign (hive formation with rubbing) was diagnostic Next Result Set

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DermAtlas, Johns Hopkins University; 2000-2005 Bernard A. Cohen, MD, Christoph U. Lehmann, MD Return to the DermAtlas Home Page
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Urticaria pigmentosa
Overview Symptoms Treatment Prevention

Definition:
A disease that produces skin lesions, intense itching, and hive formation at the site of the lesion upon rubbing.

Alternative Names:
Systemic mastocytosis; Mastocytosis; Mastocytoma

Causes, incidence, and risk factors:

Urticaria pigmentosa is caused by excessive numbers of inflammatory cells (mast cells) in the skin. It is most often seen in children, but occurs in adults as well. Rubbing of a lesion produces a rapid wheal (a hive-like bump). Younger children may develop a fluid-filled blister over a lesion if it is traumatized. A large histamine release may cause flushing, headache, diarrhea, a rapid heartbeat (tachycardia), and even fainting. This is uncommon with urticaria pigmentosa, but may be seen in other forms of mastocytosis. Urticaria pigmentosa is one of several forms of mastocytosis. Other forms include the solitary mastocytoma (a single lesion), and systemic mastocytosis (involvement in organs other than the skin).

Dermatographism close-up

Dermatographism on the arm

Urticaria pigmentosa in the armpit

Mastocytosis, diffuse cutaneous

Urticaria pigmentosa on the chest

Urticaria pigmentosa close-up

Dermatographism on the back

Review Date: 8/7/2001 Reviewed By: Michael Lehrer, M.D., Department of Dermatology, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network.

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Urticaria pigmentosa
Urticaria pigmentosa is the name given to a type of mastocytosis, in which there are brown patches on the skin due to abnormal collections of mast cells.

Mast cells are normally widely distributed over the skin. They contain granules that contain histamine and other chemicals. When the mast cell is disturbed, these chemicals are released into the surrounding skin. The chemicals make the blood vessels leaky, resulting in localised itching, swelling and redness.

Pigmented spots are seen on the back of a man with extensive urticaria pigmentosis of recent onset

Clinical features
Urticaria pigmentosa most often affects infants, with the first patches appearing at a few months of age. They are often confused with insect bites at first, but persist and gradually increase in number for several months or years. They can appear on any part of the body including the scalp, face, trunk and limbs. In urticaria pigmentosa, one can demonstrate the presence of mast cells by rubbing one of the brown patches. Within a few minutes, the rubbed area becomes reddened, swollen and itchy. This is known as Dariers sign, and confirms the presence of mastocytosis.

Urticaria pigmentosa in an infant may resemble persistent insect bites In young children, it is common for the patches to blister when rubbed. If many patches are activated at the same time the infant may become irritable but is uncommon for severe symptoms to arise.

Over the next few years the urticaria pigmentosa becomes less irritable and eventually the patches fade away. By the teenage years, most patches will have gone. Sometimes urticaria pigmentosa develops for the first time in an adult. Few or many lesions appear and can be unsightly as well as itchy. Unfortunately, in adults urticaria pigmentosa tends to persist long term. It is also more likely to be associated with internal symptoms. Mast cells can accumulate in other organs as well as the skin (systemic mastocytosis). If this occurs, it may cause fever, weight loss, diarrhoea and abdominal pain. Rarely, systemic mastocytosis is due to malignancy and results in a form of leukaemia. Affected sites may be bone, liver, spleen, lymph nodes or the gastrointestinal tract. Telangiectasia eruptiva macularis perstans (TEMP) is a rare form of mastocytosis in which diffuse red patches occur associated with overlying telangiectasia (dilated capillaries).

Precautions
Exercise or heat can aggravate symptoms. A severe reaction can result in flushing and faintness. Certain medications can cause mast cell degranulation and should be avoided if there is extensive urticaria pigmentosa. These include:

Aspirin (salicylates) Codeine and morphine (narcotics) Alcohol Anticholinergics

Tests
The appearance of urticaria pigmentosa is generally so characteristic that no specific tests are necessary. However, occasionally a skin biopsy is needed to confirm the diagnosis. If there are any symptoms suggesting internal involvement, the following tests may be helpful:

X-rays of skull and long bones may show areas of bone thinning, or a diffuse ground glass appearance Liver function blood tests but the results may be normal even with extensive liver involvement Urinary histamine levels or 1,4-methylimidazole acetic acid may be elevated

Treatment
Urticaria pigmentosa is not serious, and does not require any treatment in most cases. However the following can be helpful.

Oral antihistamines Mast cell stabilisers Disodium cromoglycate orally may be helpful in some cases

Low-dose aspirin Paradoxically, this can help some patients, but must be taken cautiously as aspirin can also worsen symptoms Topical steroids Potent steroid creams applied for several months under occlusion can reduce itching and unsightliness, but the patches tend to recur within a few months. Topical steroids are only suitable for limited areas. Photochemotherapy.(PUVA) This form of ultraviolet radiation is the most effective treatment for adults with urticaria pigmentosa. Two or three treatments each week are required for several months. PUVA lessens the itch and improves the appearance. The urticaria pigmentosa is likely to recur within six to twelve months but the treatment can be repeated. Interferon An expensive treatment appropriate only for the most severely affected individuals

Related information

Other web sites: Mastocytosis fact sheet - from National Institute of Allergy & Infectious Diseases Mastocytosis - from emedicine dermatology, the online textbook

DermNet does not provide an on-line consultation service. If you have any concerns with your skin or its treatment, see a dermatologist for advice.
Created 1997. Last updated 29 May 2005. 2005 NZDS. Disclaimer.

Illustrations

Dermatographis m - close-up

Dermatographis Urticaria m on the arm pigmentos a in the armpit

Mastocytosis Urticaria , diffuse pigmentos cutaneous a on the chest

Urticaria pigmentos a - closeup

Dermatographis m on the back

Alternative names

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Mastocytosis; Mastocytoma Definition

Return to top

Urticaria pigmentosa is a disease that produces skin lesions and intense itching. If the lesions are rubbed, hives may form on the site. Causes, incidence, and risk factors
Return to top

Urticaria pigmentosa is one of several forms of mastocytosis, which is caused by excessive numbers of inflammatory cells (mast cells) in the skin. Other forms include solitary mastocytoma (a single lesion) and systemic mastocytosis (involvement in organs other than the skin). Urticaria pigmentosa is most often seen in children, but it can occur in adults as well. Rubbing of a lesion produces a rapid wheal (a hive-like bump). Younger children may develop a fluidfilled blister over a lesion if it is scratched. A large histamine release from these extra mast cells may cause flushing, headache, diarrhea, a rapid heartbeat (tachycardia), and even fainting. This is uncommon with urticaria pigmentosa but may be seen in other forms of mastocytosis. Symptoms
Return to top

Appearance of brownish lesions on skin Welt or hive formation when lesions are rubbed or scratched Blister formation over lesion when it is rubbed Facial flushing

Severe form: Diarrhea Fast heart rate Headache Fainting (syncope) Signs and tests
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Urine histamine -- shows elevated levels of histamine Skin biopsy -- confirms the presence of increased number of mast cells
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Treatment

Antihistamines may relieve some of the histamine-induced symptoms such as itching and flushing. Discuss the choice of antihistamine with your child's health care provider. With more severe and unusual forms of urticaria pigmentosa, systemic symptoms should be controlled with other medications. Expectations (prognosis)
Return to top

Urticaria pigmentosa goes away by puberty in about half of the affected children. Symptoms usually decrease in the remaining patients as they grow into adulthood. In adults, urticaria pigmentosa may progress to the more serious systemic mastocytosis. Complications
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Discomfort from itching, and possible self-consciousness about the "spots," are the primary complications. Other problems such as diarrhea and fainting are rare. Certain medications may trigger flares of urticaria pigmentosa. Discuss these with your doctor. Bee stings may also cause severe allergic reactions in some patients with urticaria pigmentosa. Your doctor may advise you to carry an EpiPen to be used in case of a bee sting. Calling your health care provider
Return to top

Call for an appointment with your health care provider if your child has symptoms suggesting urticaria pigmentosa. Update Date: 10/29/2004
Updated by: Jonathan Kantor, M.D., Department of Dermatology, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network.
A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is the first of its kind, requiring compliance with 53 standards of quality and accountability, verified by independent audit. A.D.A.M. is among the first to achieve this important distinction for online health information and services. Learn more about A.D.A.M.'s editorial process. A.D.A.M. is also a founding member of Hi-Ethics (www.hiethics.com) and subscribes to the principles of the Health on the Net Foundation (www.hon.ch).

The information provided should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Adam makes no representation or warranty regarding the accuracy, reliability, completeness, currentness, or timeliness of the content, text or graphics. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 2005, A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

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National Organization for Rare Disorders, Inc.

Urticaria Pigmentosa
Important It is possible that the main title of the report Urticaria Pigmentosa is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report. Synonyms
Localized Infantile Mastocytosis Mastocytosis, Infantile Nettleship's, E. Disease Type I Urticaria, Perstans Hemorrhagica Xanthelasmoidea
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Topic Contents

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General Discussion Urticaria pigmentosa is a rare skin disorder that is a localized (cutaneous) form of mastocytosis. Some clinicians suggest that urticaria pigmentosa is the childhood form of mastocytosis. Mast cells are specialized cells of connective tissue that release substances such as histamine (a chemical important in the inflammatory process) and heparin (an anti-clotting agent) when the bodys alarm mechanism is set off. When mast cells cluster and multiply excessively (proliferate), histamine and heparin are released into the skin (mastocytosis). The characteristic skin lesions of urticaria pigmentosa appear in these areas. Urticaria pigmentosa is generally benign and is usually self-limited. The exact cause of the disease is not known, although some cases may be inherited.

. Resources NIH/National Institute of Allergy and Infectious Diseases 9000 Rockville Pike Building 31A Bethesda, MD 20892 Tel: (301)496-5717 Fax: (301)402-0120 Internet: http://www.niaid.nih.gov/ American Academy of Allergy Asthma and Immunology 611 East Wells Street Milwaukee, WI 53202 Tel: (414)272-6071 Fax: (414)276-3349 Tel: (800)822-2762 Email: info@aaaai.org Internet: http://www.aaaai.org For a Complete Report This is an abstract of a report from the National Organization for Rare Disorders, Inc. (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians. It is possible that the title of this topic is not the name you selected. Please check the

Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report. This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org Last Updated: 3/18/2003 Copyright 1992, 1994, 2003 National Organization for Rare Disorders, Inc.
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Overview

Symptoms

Treatment

Prevention

Urticaria pigmentosa
Alternative Names: Mastocytosis; Mastocytoma

Treatment: Antihistamines may relieve some of the histamine-induced symptoms such as itching a flushing. Discuss the choice of antihistamine with your child's health care provider. Wit more severe and unusual forms of urticaria pigmentosa, systemic symptoms may need be controlled with other medications. Expectations (prognosis): Urticaria pigmentosa goes away by puberty in about half of the affected children. Symptoms usually decrease in the remaining patients as they grow into adulthood.

In adults, urticaria pigmentosa may progress to the more serious systemic mastocytos

Complications: Discomfort from itching, and possible self-consciousness about the "spots," are the primary complications. Other problems such as diarrhea and fainting are rare.

Certain medications may trigger flares of urticaria pigmentosa. Discuss these with you doctor.

Bee stings may also cause severe allergic reactions in some patients with urticaria pigmentosa. Your doctor may advise you to carry an EpiPen to be used in case of a be sting.

Calling your health care provider: Call for an appointment with your health care provider if your child has symptoms suggesting urticaria pigmentosa.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is the first of its kind, requiring compliance with 53 standards of quality and accountability, verified by independent audit. A.D.A.M. is among the first to achieve this important distinction for online health information services. Learn more about A.D.A.M.'s editorial reviewers. A.D.A.M. is also a founding mem Hi-Ethics (www.hiethics.com) and subscribes to the principles of the Health on the Net Foundation (www.hon.ch).

The information provided herein should not be used during any medical emergency or the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for al medical emergencies. Links to other sites are provided for information only -- they do n constitute endorsements of those other sites. Copyright 2004 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

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Urticaria pigmentosa
Alternative Names
Mastocytosis; Mastocytoma

Treatment
Antihistamines may relieve some of the histamine-induced symptoms such as itching and flushing. Discuss the choice of antihistamine with your child's health care provider. With more severe and unusual forms of urticaria pigmentosa, systemic symptoms should be controlled with other medications.

Outlook (Prognosis)
Urticaria pigmentosa goes away by puberty in about half of the affected children. Symptoms usually decrease in the remaining patients as they grow into adulthood. In adults, urticaria pigmentosa may progress to the more serious systemic mastocytosis.

Possible Complications
Discomfort from itching, and possible self-consciousness about the "spots," are the primary complications. Other problems such as diarrhea and fainting are rare. Certain medications may trigger flares of urticaria pigmentosa. Discuss these with your doctor. Bee stings may also cause severe allergic reactions in some patients with urticaria pigmentosa. Your doctor may advise you to carry an EpiPen to be used in case of a bee sting.

When to Contact a Medical Professional

Call for an appointment with your health care provider if your child has symptoms suggesting urticaria pigmentosa.

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Mastocytosis, diffuse cutaneous

Urticaria pigmentosa on the chest

Urticaria pigmentosa close-up

Dermatographism on the back

Review Date: 10/29/2004 Reviewed By: Jonathan Kantor, M.D., Department of Dermatology, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network.
A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is the first of its kind, requiring compliance with 53 standards of quality and accountability, verified by independent audit. A.D.A.M. is among the first to achieve this important distinction for online health information and services. Learn more about A.D.A.M.'s editorial process. A.D.A.M. is also a founding member of Hi-Ethics (www.hiethics.com) and subscribes to the principles of the Health on the Net Foundation (www.hon.ch).
The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright duplication or distribution of the information contained herein is strictly prohibited. 2005A.D.A.M., Inc. Any

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A B C D E F G H I J K L M N O P Q R S T U VWX Y Z

Dermatographism - close-up

Dermatographism on the arm

Urticaria pigmentosa in the armpit

Mastocytosis, diffuse cutaneous

Urticaria pigmentosa on the chest

Urticaria pigmentosa close-up

Dermatographism on the back

Overview

Symptoms

Treatment

Prevention

Urticaria pigmentosa
Alternative Names: Mastocytosis; Mastocytoma Treatment: Antihistamines may relieve some of the histamine-induced symptoms such as itching and flushing. Discuss the choice of antihistamine with your child's health care provider. With more severe and unusual forms of urticaria pigmentosa, systemic symptoms should be controlled with other medications.

Expectations (prognosis): Urticaria pigmentosa goes away by puberty in about half of the affected children. Symptoms usually decrease in the remaining patients as they grow into adulthood. In adults, urticaria pigmentosa may progress to the more serious systemic

mastocytosis.

Complications: Discomfort from itching, and possible self-consciousness about the "spots," are the primary complications. Other problems such as diarrhea and fainting are rare. Certain medications may trigger flares of urticaria pigmentosa. Discuss these with your doctor. Bee stings may also cause severe allergic reactions in some patients with urticaria pigmentosa. Your doctor may advise you to carry an EpiPen to be used in case of a bee sting.

Calling your health care provider: Call for an appointment with your health care provider if your child has symptoms suggesting urticaria pigmentosa.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is the first of its kind, requiring compliance with 53 standards of quality and accountability, verified by independent audit. A.D.A.M. is among the first to achieve this important distinction for online health information and services. Learn more about A.D.A.M.'s editorial process. A.D.A.M. is also a founding member of Hi-Ethics (www.hiethics.com) and subscribes to the principles of the Health on the Net Foundation (www.hon.ch).

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 2004 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.