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  3. Disseminated superficial actinic porokeratosis
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Disseminated superficial actinic porokeratosis


Title




Other Names:
DSAP
Categories:
Congenital and Genetic Diseases; Skin Diseases

Summary Summary


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Disseminated superficial actinic porokeratosis (DSAP) is a skin condition that causes dry, scaly patches.[1] Symptoms include a large number of small, brownish patches with a distinctive border, found most commonly on sun-exposed areas of the skin (particularly the lower arms and legs).[1][2] DSAP usually starts during the third or fourth decade of life and rarely affects children. Lesions generally are more prominent in the summer and less prominent in the winter. While DSAP is usually not cancerous, squamous cell carcinoma or Bowen’s disease may occasionally develop within patches.[3] DSAP may be inherited in an autosomal dominant matter or may occur in people with no family history of DSAP.[3] Some cases are caused by a change (variant) in the MVK or SART3 genes.[3][4] There is no standard treatment for DSAP, and treatment is generally not effective long-term.[1][2] Sun avoidance may reduce the development of new patches.[1] Treatments that have been beneficial in some people include topical imiquimod cream, topical 5-fluorouracil, and topical vitamin D analogs such as tacalcitol and calcipotriol. A newer therapy that has been successful is topical cholesterol/lovastatin.[5] Other therapies that have been tested with varying results include cryotherapy, electrodessication (using electrical currents to remove patches), laser ablation, and photodynamic therapy.[1][2][3]
Last updated: 2/15/2021

Symptoms Symptoms


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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.

Showing of 4 |
Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Porokeratosis 0200044
30%-79% of people have these symptoms
Cutaneous photosensitivity
Photosensitive skin
Photosensitive skin rashes
Photosensitivity
Sensitivity to sunlight
Skin photosensitivity
Sun sensitivity
[ more ]
0000992
5%-29% of people have these symptoms
Pruritus
Itching
Itchy skin
Skin itching
[ more ]
0000989
Squamous cell carcinoma 0002860
Showing of 4 |
Do you have more information about symptoms of this disease? We want to hear from you.
Last updated: 2/1/2021
Do you have updated information on this disease? We want to hear from you.

Related Diseases Related Diseases


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Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.

Conditions with similar signs and symptoms from Orphanet
Differential diagnoses include (pre)neoplastic or hyperplastic keratotic skin lesions as well as other forms of porokeratosis, such as porokeratosis of Mibelli or superficial disseminated porokeratosis (similar to DSAP but not triggered by sunlight).
Visit the Orphanet disease page for more information.

Organizations Organizations


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Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

  • British Association of Dermatologists
    19 Fitzroy Square
    London, UK W1T 6EH
    United Kingdom
    Telephone: 0207 383 0266
    Fax: 0207 388 5263
    E-mail: admin@bad.org.uk
    Website: http://www.bad.org.uk
  • Genetic Skin Disease Center
    Stanford Medical Dermatology Clinic
    Stanford Medicine Outpatient Center
    450 Broadway
    Pavilion B, 4th Floor
    Redwood City, CA 94063
    Telephone: 650-723-6316
    Fax: 650-725-7711
    Website: http://dermatology.stanford.edu/contact/

Social Networking Websites

  • Visit the following Facebook groups related to Disseminated superficial actinic porokeratosis:
    DSAP Institute Private Forum (Closed Group)

Organizations Providing General Support

  • American Academy of Dermatology
    1445 New York Ave, NW
    Suite 800
    Washington, DC 20005
    Toll-free: 888-462-DERM (3376)
    Fax: 847-240-1859
    E-mail: https://www.aad.org/Forms/ContactUs/Default.aspx
    Website: https://www.aad.org/
  • Skin Support
    Website: http://www.skinsupport.org.uk/
Do you know of an organization? We want to hear from you.

Learn More Learn More


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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

  • The British Association of Dermatologists has a patient information leaflet about Disseminated superficial actinic porokeratosis.
  • 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.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Disseminated superficial actinic porokeratosis. Click on the link to view a sample search on this topic.

Selected Full-Text Journal Articles

  • Rouhani P, Fischer M, Meehan S, Keltz Pomeranz M. Disseminated superficial actinic porokeratosis. Dermatology Online Journal. December 2012;18(12):24.

News & Events News & Events


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News

  • Rare Disease Day at NIH 2021
    March 1, 2021

GARD Answers GARD Answers


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Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

Have a question? Contact a GARD Information Specialist.

References References


  1. Disseminated superficial actinic porokeratosis. DermNet NZ. April, 2017; https://www.dermnetnz.org/topics/disseminated-superficial-actinic-porokeratosis/.
  2. Kanitakis J. Disseminated superficial actinic porokeratosis. Orphanet. October, 2015; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=79152.
  3. Isabelle Touitou et. al. The expanding spectrum of rare monogenic autoinflammatory diseases. Orphanet Journal of Rare Diseases. 2013; 8:162:http://www.ojrd.com/content/8/1/162.
  4. Linda V Spencer. Porokeratosis. Medscape. August 7, 2017; http://emedicine.medscape.com/article/1059123-overview.
  5. Atzmony L, Lim YH, Hamilton C, Leventhal JS, Wagner A et al. Topical cholesterol/lovastatin for the treatment of porokeratosis: A pathogenesis-directed therapy. J Am Acad Dermatol. Jan 2020; 82(1):123-131. https://pubmed.ncbi.nlm.nih.gov/31449901.
Do you know of a review article? We want to hear from you.
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