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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.
- Localized Infantile Mastocytosis
- Mastocytosis, Infantile
- Nettleship's, E. Disease Type I
- Urticaria, Perstans Hemorrhagica
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 body's 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.
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For a Complete Report
This is an abstract of a report from the National Organization for Rare Disorders (NORD). A copy of the complete report can be downloaded free from the NORD website for registered users. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational therapies (if available), and references from medical literature. For a full-text version of this topic, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".
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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.
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Last Updated: 12/16/1969
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