National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Atrophoderma of Pasini and Pierini


Other Names:
Idiopathic atrophoderma of Pasini and Pierini; Congenital atrophoderma of Pasini and Pierini
Categories:
Atrophoderma of Pasini and Pierini (ATPP) is a rare skin condition that results in patchy areas of discolored skin. The main symptom of atrophoderma of Pasini and Pierini is multiple irregularly shaped, dark or light areas of skin in which the tissue under the skin breaks down resulting in a depression (dent). Symptoms usually start in adolescence or early adulthood and can progress for 10-20 years. The cause of ATPP is unknown. Diagnosis is based on clinical examination and a skin biopsy. There is no single specific treatment for this rare condition.[1][2]
Last updated: 5/27/2020
The following list includes the most common signs and symptoms in people with atrophoderma of Pasini and Pierini. These features may be different from person to person. Some people may have more symptoms than others and symptoms can range from mild to severe. This list does not include every symptom or feature that has been described in this condition.

The main symptom of atrophoderma of Pasini and Pierini is dark or light-colored indented areas of skin with irregular shapes and borders. Some people have pain, itching, or numbness associated with the skin findings.[1][2] 

The skin findings in this condition usually appear in adolescence or early adulthood. The trunk is usually affected first and then the changes in the skin may spread to the chest, arms and stomach. Atrophoderma of Pasini and Pierini may progress over 10-20 years and then stops.  Very rarely, this condition will progress to systemic sclerosis.[2][3] 
Last updated: 5/27/2020
The cause of atrophoderma of Pasini and Pierini is unknown. In some cases, a bacteria known as Borrelia burgdorferi may be the cause. In others, a possible genetic cause is suspected.[1][2] 
Last updated: 5/27/2020
Atrophoderma of Pasini and Pierini is diagnosed based on a clinical examination and a skin biopsy to look at a piece of affected skin under the microscope.[2][4]
Last updated: 5/27/2020
There is no specific treatment for atrophoderma of Pasini and Pierini.  Treatments that have been tried with some success include antibiotics, topical medications, anti-malarial drugs, and laser treatments.[1][5]

Specialists who may be involved in the care of someone with atrophoderma of Pasini and Pierini include:
  • Dermatologist
  • Pharmacist
Last updated: 5/27/2020
The exact number of people with atrophoderma of Pasini and Pierini is unknown. It is extremely rare, and less than 100 cases have been reported in the literature.[2][4]
Last updated: 5/27/2020

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • The Scleroderma Clinical Trials Consortium is an international organization of scleroderma clinical researchers. The consortium Web site contains a listing of active scleroderma trials, past copies of the Scleroderma Care and Research journal, and a tool for finding your nearest member institution. 

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

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.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Atrophoderma of Pasini and Pierini. Click on the link to view a sample search on this topic.

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  1. Litaiem N, Idoudi S. Atrophoderma of Pasini and Pierini. In: StatPearls. Treasure Island (FL): StatPearls Publishing. Jan 2019; https://pubmed.ncbi.nlm.nih.gov/30085611.
  2. Muntyanu A, Rdpath M, Roshdy O, Jfri A. Idiopathic Atrophoderma of Pasini and Pierini: Case report and literature review. Clin Case Rep. 2018; 7(2):258-263. https://pubmed.ncbi.nlm.nih.gov/30847185.
  3. Bassi A, Remaschi G, Difonzo EM, et la. Idiopathic congenital atrophoderma of Pasini and Pierini. Arch Dis Child. 2015; 100(12):1184. https://pubmed.ncbi.nlm.nih.gov/26374755/.
  4. Begolli Gerqari A, Ferizi M, Gerqari I. Atrophoderma of Pasini and Pierini in a young adult: a case report. Acta Dermatovenerol Alp Pannonica Adriat. 2019; 28(1):45-46. https://pubmed.ncbi.nlm.nih.gov/30901070.
  5. Honda M, Oyama N, Uesugi S, Katoh T, Hasegawa M. Successful treatment with oral minocycline in a case with a long-standing idiopathic atrophoderma of Pasini and Pierini: Histopathological comparison with the contralateral normal skin. J Dermatol. 2019; 46(12):e472-e474. https://pubmed.ncbi.nlm.nih.gov/31463968.