Erythema Dyschromicum Perstans 

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD   more...
 
Updated: May 2, 2011
 

Background

C. Oswaldo Ramirez of San Salvador, El Salvador, first described erythema dyschromicum perstans (EDP) in 1957.[1] He called the patients with this eruption Los cenicientos, meaning the ashen ones. The Spanish term cenicienta also means Cinderella because of this folklore character's close association with ashes from sitting at home alone by the fireplace. Later, erythema dyschromicum perstans was called dermatosis ceniciento, meaning ashy dermatosis, because of its ashy bluish gray color. The term erythema dyschromicum perstans is credited to Marion B. Sulzberger, who suggested it when examining Convit's[2] patients in Caracas. Sulzberger's comment, in discussion of another paper, is as follows:

... the narrow red border (which is often hard to find), represents the active lesions. This is why I suggested a name which contains the term "erythema" and which also suggests the variety and persistence of the final dyschromias.

The descriptive term ashy dermatosis was also used as a designation for their coloration. In South America, another name, erythema chronicum figuratum melanodermicum, is also used.

Erythema dyschromicum perstans (ashy dermatosis) is a distinct and somewhat controversial cutaneous eruption that may be best regarded as a form of lichen planus or lichen planus actinicus.[3, 4, 5]

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Pathophysiology

The etiology of erythema dyschromicum perstans is unknown, but many consider erythema dyschromicum perstans to be a variant of lichen planus actinicus. A variety of predisposing factors have been cited. These include ingestion of ammonium nitrite, an intestinal parasitosis caused by nematodes (whipworm infection, control of which produced erythema dyschromicum perstans remission), orally administered radiographic contrast media, and, possibly, an occupationally associated cobalt allergy in a plumber. One case may be particularly revealing, that of a 13-year-old rural northern European truant who repeatedly ingested small amounts of a fertilizer, ammonium nitrate, to induce erythema dyschromicum perstans and avoid school. Chlorothalonil exposure among banana farm workers is another possible cause of erythema dyschromicum perstans.[6]

An abnormality in cell-mediated immunity might play a role. However, substantial immune dysfunction is limited at present to 1 report of an HIV-seropositive 41-year-old homosexual of Chinese lineage with erythema dyschromicum perstans.[7, 8]

HLA-DR association with the genetic susceptibility to develop ashy dermatosis in Mexican Mestizo patients was analyzed, the results of which were reported by Correa in 2007.[9] The most frequent allele was HLA-DR4 (65%), compared with 23% in controls. These Mexican patients had HLA-DR4 subtype *0407, which is also found in the Amerindian population.[10, 11] Thus, although many factors may be involved, an important genetic susceptibility appears to be conferred by genes located within the major histocompatibility complex region.

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Epidemiology

Frequency

International

Erythema dyschromicum perstans is most common in Latin America and Asia; most of the cases occur in El Salvador where the first case was identified. Cases in Europe have also been described, including in Italy.[12]

Mortality/Morbidity

Erythema dyschromicum perstans has a benign outcome, with most complaints relating to cosmetic issues.

Race

Darker-skinned individuals seem to be affected more often than lighter-skinned individuals. It is a rare disorder of pigmentation that is most common in Hispanic patients.[13] Unlike adult patients, who are most commonly of Hispanic origin, children with erythema dyschromicum perstans are usually white.[14]

Sex

Both sexes are affected, but women are affected more often than men.

Age

The age range affected is wide, both in Latin America and around the world. Erythema dyschromicum perstans has been observed in children aged 1 year and adults aged 80 years. For example, a 6-year-old British girl and 4 Finnish children aged 8-12 years were described, as were many older patients. An early report had a series of 5 patients; 3 males aged 11-36 years and 2 women.

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Contributor Information and Disclosures
Author

Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

Santiago A Centurion, MD  Staff Physician, Department of Dermatology, New Jersey Medical School, University of Medicine and Dentistry of New Jersey

Santiago A Centurion, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, and Sigma Xi

Disclosure: Nothing to disclose.

Specialty Editor Board

Shyam Verma, MBBS, DVD, FAAD  Adjunct Clinical Assistant Professor, Department of Dermatology, University of Virginia, State University of New York at Stonybrook, Penn State University

Shyam Verma, MBBS, DVD, FAAD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD  Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and American College of Rheumatology

Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Consulting fee Consulting; Celgene Honoraria Safety Monitoring Committee; GSK - Glaxo Smith Kline Consulting fee Consulting; TenXBioPharma Consulting fee Safety Monitoring Committee

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References

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Ash-colored, partially confluent, macular lesions over the patient's back. Reprint with permission from Cutis 1986; 37: 42-44.
Close-up photograph shows ash-colored macular lesions and lack of an inflammatory border. Reprint with permission from Cutis 1986; 37: 42-44.
Photomicrograph of a lesion on the patient's back shows slight basilar vacuolar change, extensive pigment incontinence, dilatation of dermal lymphatics, and lack of inflammation (hematoxylin & eosin, original magnification X24.8). Reprint with permission from Cutis 1986; 37: 42-44.
Higher-power photograph shows slight vacuolar basilar change, marked dilatation of intradermal lymphatics, incontinence of melanin pigment, and lack of inflammation (hematoxylin & eosin, original magnification X238). Reprint with permission from Cutis 1986; 37: 42-44.
 
 
 
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