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Genetic and Rare Diseases Information Center (GARD)

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Morphea


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Your Question

What is morphea and atrophoderma of Pierini and Pasini? How is atrophoderma of Pierini and Pasini treated?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is morphea?

Morphea (mor-FEE-ah) comes from a Greek word that means “form” or “structure.” The word refers to local patches of scleroderma. The first signs of the disease are reddish patches of skin that thicken into firm, oval-shaped areas. The center of each patch becomes ivory colored with violet borders. These patches sweat very little and have little hair growth. Patches appear most often on the chest, stomach, and back. Sometimes they appear on the face, arms, and legs.[1] In most cases, morphea improves spontaneously over time (typically 3 to 5 years); however, patients are often left with patches of darkened or discolored skin and, in rare cases, muscle damage.[1][2] The cause of this condition is unknown.[3] Morphea can be either localized or generalized.[1]
Last updated: 1/28/2010

What is atrophoderma of Pierini and Pasini?

Atrophoderma of Pierini and Pasini is thought to possibly represent a late stage of morphea a type of localized scleroderma. Signs and symptoms of atrophoderma of Pierini and Pasini include multiple oval, darkened (hyperpigmented) plaques in which tissue under the skin breaks down so that there is a depression (dent) within the skin.[4] Some findings suggest that atrophoderma of Pierini and Pasini may be associated with B burgdorferi, a bacteria that causes Lyme disease, in some cases.[5]
Last updated: 1/21/2010

How might atrophoderma of Pierini and Pasini be treated?

No single treatment of atrophoderma of Pierini and Pasini is consistently effective. Therapies that have been tried include topical corticosteroids, antibiotics, and antimalarials. There have been reports of symptom improvement with the use of hydroxychloroquine, potassium aminobenzoate, and improvement following surgical care using a Q-switched alexandrite laser, however these findings have not been confirmed by larger studies.[5] If a person with atrophoderma of Pierini and Pasini tests positive for B burgdorferi antibody, standard Lyme disease therapy is often recommended. Click here to visit the Center for Disease Control and Prevention's Lyme Disease Information Resource pages to learn more about Lyme disease therapy.
Last updated: 1/21/2010

Are there any research studies investigating new therapies for treatment of atrophoderma of Pierini and Pasini?

We did not find clinical trials specific to atrophoderma, however there is a study titled  Evaluation and Treatment of Patients with Dermatologic Diseases that is enrolling people with skin conditions in general, and may be of interest to you. Click on the study title to learn more.

In addition, The Research Portfolio Online Reporting Tool (RePORT) provides access to reports, data, and analyses of research activities at the National Institutes of Health (NIH). There is a study titled Dermatology Consultation Clinic and Clinical Research that may be of interest to you. You may want to contact the investigator, Maria Turner (maria.turner@nih.gov) to learn more.

You can also contact the Patient Recruitment and Public Liaison (PRPL) Office at the National Institutes of Health (NIH). We recommend calling the toll-free number listed below to speak with a specialist, who can help you determine if you are eligible for any clinical trials.

Patient Recruitment and Public Liaison Office
NIH Clinical Center
Bethesda, Maryland 20892-2655
Toll-free: 800-411-1222
Fax: 301-480-9793
Email: prpl@mail.cc.nih.gov
Web site:  http://clinicalcenter.nih.gov/ 

If you are interested in enrolling in a clinical trial, you can find helpful general information on clinical trials at the following ClinicalTrials.gov Web page.
http://clinicaltrials.gov/ct2/info/understand 

Resources on many charitable or special-fare flights to research and treatment sites and low-cost hospitality accommodations for outpatients and family members, as well as ambulance services, are listed on the Web site of the Office of Rare Diseases Research (ORDR), part of the National Institutes of Health.
http://rarediseases.info.nih.gov/Resources.aspx?PageID=8 

Last updated: 9/20/2010

References
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.