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Disease Profile

Pigmented purpuric dermatosis

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

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Europe Estimated

Age of onset

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ICD-10

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Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Schamberg disease; Familial pigmented purpuric eruption; Schamberg purpura;

Categories

Skin Diseases

Summary

Pigmented purpuric dermatosis is a chronic condition characterized by reddish-brown skin lesions caused by leaky capillaries. Although they can arise on any part of the body, they are most commonly located on the lower legs.[1][2] In some cases, the skin lesions cause severe itching. The skin lesions may spread over time, or clear up on their own.[1] The cause of pigmented purpuric dermatosis is unknown. While treatment may not be necessary, topical steroids may help control itching. Other options for management include compression stockings, oral medications, and phototherapy.[1][2]

Symptoms

The main features of pigmented purpuric dermatosis (PPD) are petechiae (tiny red spots due to broken blood vessels) or purpura (purple-colored spots or patches due to broken blood vessels), and yellow to brown pigmented patches. The most common sites are the legs, although lesions also may develop in other areas. Infrequently, PPD manifests as a generalized rash. The palms of the hands, soles of the feet, genitalia, and mucosa typically are not affected. Depending on the type of PPD a person has, additional signs and symptoms may include red-brown patches, plaques, linear lesions, itching, and/or other features. In general, PPD does not cause serious health problems but is a chronic condition. Signs and symptoms may persist, wax and wane, or slowly progress, and may go away over a period of months to years.[3]

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Medical Terms Other Names
Learn More:
HPO ID
Percent of people who have these symptoms is not available through HPO
Abnormality of the skin
0000951
Autosomal dominant inheritance
0000006
Neonatal onset
0003623
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Cause

The cause of pigmented purpuric dermatosis is unknown.[1][2] Occasionally, it occurs as a reaction to a medication, food additive, viral infection or following exercise.[2] In rare cases, there appears to be a genetic component.[1]

Treatment

If the condition is mild, treatment may not be necessary. For those who suffer from itching, topical steroids and antihistamines may be useful. Compression stockings may be used if the lower legs are affected.[1][2] Additional treatments that have been successful in limited studies include pentoxifylline,[4] aminaphtone,[5] photochemotherapy (PUVA)[6], griseofulvin, oral cyclosporin, and ascorbic acid with rutoside.[7]

Learn more

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Pigmented purpuric dermatosis. Click on the link to view a sample search on this topic.

References

  1. Mehregan D. Pigmented Purpuric Dermatitis. eMedicine Journal. March 31, 2016; https://emedicine.medscape.com/article/1084594-overview.
  2. Capillaritis. DermNet New Zealand. January 2016; https://www.dermnetnz.org/topics/capillaritis/.
  3. Garg A. Pigmented purpuric dermatoses (capillaritis). UpToDate. Waltham, MA: UpToDate; September, 2019; https://www.uptodate.com/contents/pigmented-purpuric-dermatoses-capillaritis.
  4. Gandhi V, Singal A, Sachdeva B, Bhattacharya SN. Treatment of Schamberg's disease with pentoxifylline--therapeutic trial. Indian J Dermatol Venereol Leprol. Jan-Feb 2003; 69(1):25-26. https://www.ncbi.nlm.nih.gov/pubmed/17642818.
  5. de Godoy JM, Batigália F. Aminaphtone in the control of Schamberg's disease. Thromb J. June 2009; 7:8. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2703626/.
  6. Seckin D, Yazici Z, Senol A, Demircay Z. A case of Schamberg's disease responding dramatically to PUVA treatment. Photodermatol Photoimmunol Photomed. April 2008; 24(2):95-96. https://www.ncbi.nlm.nih.gov/pubmed/18353090.
  7. Schober SM, Peitsch WK, Bonsmann G, Metze D, Thomas K, Goerge T, Luger TA, Schneider SW. Early treatment with rutoside and ascorbic acid is highly effective for progressive pigmented purpuric dermatosis. J Dtsch Dermatol Ges. Dec 2014; 12(12):1112-9. https://www.ncbi.nlm.nih.gov/pubmed/25482694.

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