Atrophoderma of Pasini and Pierini


© Shannon Evans

First labeled in 1923, Atrophoderma of Pasini and Pierini was described as a bluish, depressed skin lesion that had a "cliff-drop" border. Another description commonly used is "footprints in the snow" characterizing the common oval shape of the depressed lesions, http://tray.dermatology.uiowa.edu/Atroph... . Even seventy-eight years after clinical identification, very little about Atrophoderma of Pasini and Pierini is yet understood.

WHAT IS ATROPHODERMA OF PASINI AND PIERINI
According to the Dorland's Illustrated Medical Dictionary, Atrophoderma of Pasini and Pierini is clinically defined as, "a condition most commonly occurring on the trunk, especially the back, of young women, characterized by the development of soft, bluish-brown to violaceous [bluish-purple] atrophic plaques with central induration that resemble the lesions of the late stages of morphea."

A morphea results from an overproduction of collagen deposits forming hard, callused lesions. Morphea, or localized scleroderma, has several different forms varying in severity, appearance and location including generalized morphea, deep morphea, linear morphea and plaque morphea. Atrophoderma of Pasini and Pierini falls into the plaque morphea category based on its horizontal, oval plaques. It is generally benign and has little inflammation and/or hardening at the lesion site.

However, disagreement among the medical community as to whether or not Atrophoderma of Pasini and Pierini should be classified as a type of morphea or a separate individual disorder remains at the forefront of understanding this disease. Atrophoderma of Pasini and Pierini is listed at the Office of Rare Diseases, http://rarediseases.info.nih.gov/ord/dis... , as a rare condition affecting less than 200,000 Americans.

CAUSES
The exact cause of Atrophoderma of Pasini and Pierini remains unknown. A genetic relationship has yet to be accurately determined, although some patients have reported a family history of the condition. If it is indeed a type of morphea, several causes, (according to an article written by Dr. Michael Girardi and Dr. Julie Schaffer of Yale University School of Medicine published at Emedicine, http://www.emedicine.com/derm/topic272.h... ), have been suggested for this disease including:

1. Side effects of radiation therapy
2. Various viruses (chicken pox, measles, Borrelia burgdorfei)
3. Vaccines (tetanus and bacillus Calmette-Guerin)
4. Trauma

TREATMENTS
Typical treatments for Atrophoderma of Pasini and Pierini involve powerful steroid medication applied topically or injected into the lesion. However, these treatments have not proven successful by any means. Topical Calcipotriene has shown promise as has Q-switched alexandrite laser therapy (Search Dr. CJ Arpey, et al at PubMed for more information on laser therapy). These treatments would definitely be worth mentioning to your doctor.

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