eMedicine Specialties > Dermatology > Reactive & Inflammatory Dermatoses

Erythema Dyschromicum Perstans

Author: Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Coauthor(s): Santiago A Centurion, MD, Staff Physician, Department of Dermatology, New Jersey Medical School, University of Medicine and Dentistry of New Jersey
Contributor Information and Disclosures

Updated: May 20, 2008

Introduction

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, EDP was called dermatosis ceniciento, meaning ashy dermatosis, because of its ashy bluish gray color. The term EDP is credited to Marion B. Sulzberger, who suggested it when examining Convit's2 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.

EDP (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 (see Lichen Planus).3,4,5

Pathophysiology

The etiology of EDP is unknown, but many consider EDP 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 EDP 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 EDP and avoid school. Chlorothalonil exposure among banana farm workers is another possible cause of EDP.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 EDP.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%), an impressive association because, in controls, it was seen in only 23%. Thus, although many factors may be involved, an important genetic susceptibility appears to be conferred by genes located within the major histocompatibility complex region.

Frequency

International

EDP 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.

Mortality/Morbidity

EDP 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.10 Unlike adult patients, who are most commonly of Hispanic origin, children with EDP are usually white.11

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. EDP 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.

The Medscape Pediatric Dermatology Resource Center may be of interest.

Clinical

History

EDP has a slow onset and is unlikely to resolve spontaneously.10 The clinical course of childhood (prepubertal) may differ from that of adults; EDP may be more likely to resolve within 2-3 years.

  • EDP is an asymptomatic eruption of oval, polycyclic, or irregularly shaped, gray-blue hyperpigmented macules on the trunk, the arms, the face, and the neck.
  • It begins as ash-colored macules, sometimes with an erythematous or elevated border (see Media File 1).
  • No systemic symptoms or associations exist.

Physical

  • EDP has asymptomatic, gray-blue hyperpigmented patches of variable shape and size and an elevated erythematous border in the early stages (see Media File 2).
  • The eruption is symmetrically distributed on the face, the trunk, and the upper extremities. The oral cavity and the genitals are spared.

Causes

The etiology of EDP remains unknown.

  • It has been associated with ingestion of ammonium nitrate and whipworm infestation, but a specific etiology has not been established in any instance.
  • The geographical distribution of the disease has led some authors to suggest environmental factors, but all attempts to find these pollutants have failed.

More on Erythema Dyschromicum Perstans

Overview: Erythema Dyschromicum Perstans
Differential Diagnoses & Workup: Erythema Dyschromicum Perstans
Treatment & Medication: Erythema Dyschromicum Perstans
Follow-up: Erythema Dyschromicum Perstans
Multimedia: Erythema Dyschromicum Perstans
References
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References

  1. Ramirez CO. Los cenicientos: Problema Clinica. Memoria del Primer Congresso Centroamericano de Dermatologica,. 1957;122-130.

  2. Convit J, Kerdel-Vegas F. Erythema dyschromicum perstans a hitherto undescribed skin disease. J Invest Dermatol. 1961;36:457-62.

  3. Berger RS, Hayes TJ, Dixon SL. Erythema dyschromicum perstans and lichen planus: are they related?. J Am Acad Dermatol. Aug 1989;21(2 Pt 2):438-42. [Medline].

  4. Kark EC, Litt JZ. Ashy dermatosis--a variant of lichen planus?. Cutis. Jun 1980;25(6):631-3. [Medline].

  5. Naidorf KF, Cohen SR. Erythema dyschromicum perstans and lichen planus. Arch Dermatol. Sep 1982;118(9):683-5. [Medline].

  6. Penagos H, Jimenez V, Fallas V, O'Malley M, Maibach HI. Chlorothalonil, a possible cause of erythema dyschromicum perstans (ashy dermatitis). Contact Dermatitis. Oct 1996;35(4):214-8. [Medline].

  7. Molinero J, Vilata JJ, Nagore E, Obón L, Grau C, Aliaga A. Ashy dermatosis in an HIV antibody-positive patient. Acta Derm Venereol. Jan-Feb 2000;80(1):78-9. [Medline].

  8. Nelson MR, Lawrence AG, Staughton RC, Gazzard BG. Erythema dyschromicum perstans in an HIV antibody-positive man. Br J Dermatol. Dec 1992;127(6):658-9. [Medline].

  9. Correa MC, Memije EV, Vargas-Alarcón G, Guzmán RA, Rosetti F, Acuña-Alonzo V, et al. HLA-DR association with the genetic susceptibility to develop ashy dermatosis in Mexican Mestizo patients. J Am Acad Dermatol. Apr 2007;56(4):617-20. [Medline].

  10. Silverberg NB, Herz J, Wagner A, Paller AS. Erythema dyschromicum perstans in prepubertal children. Pediatr Dermatol. Sep-Oct 2003;20(5):398-403. [Medline].

  11. Torrelo A, Zaballos P, Colmenero I, Mediero IG, de Prada I, Zambrano A. Erythema dyschromicum perstans in children: a report of 14 cases. J Eur Acad Dermatol Venereol. Jul 2005;19(4):422-6. [Medline].

  12. Mizukawa Y, Shiohara T. Fixed drug eruption presenting as erythema dyschromicum perstans: a flare without taking any medications. Dermatology. 1998;197(4):383-5. [Medline].

  13. Volz A, Metze D, Böhm M, Bruckner-Tuderman L, Nashan D. Idiopathic eruptive macular pigmentation in a 7-year-old girl: case report and discussion of differences from erythema dyschromicum perstans. Br J Dermatol. Oct 2007;157(4):839-40. [Medline].

  14. Vega ME, Waxtein L, Arenas R, Hojyo T, Dominguez-Soto L. Ashy dermatosis and lichen planus pigmentosus: a clinicopathologic study of 31 cases. Int J Dermatol. Feb 1992;31(2):90-4. [Medline].

  15. Arbiser JL, Moschella SL. Clofazimine: a review of its medical uses and mechanisms of action. J Am Acad Dermatol. Feb 1995;32(2 Pt 1):241-7. [Medline].

  16. Bahadir S, Cobanoglu U, Cimsit G, Yayli S, Alpay K. Erythema dyschromicum perstans: response to dapsone therapy. Int J Dermatol. Mar 2004;43(3):220-2. [Medline].

  17. Baranda L, Torres-Alvarez B, Cortes-Franco R, Moncada B, Portales-Perez DP, Gonzalez-Amaro R. Involvement of cell adhesion and activation molecules in the pathogenesis of erythema dyschromicum perstans (ashy dermatitis). The effect of clofazimine therapy. Arch Dermatol. Mar 1997;133(3):325-9. [Medline].

  18. Combemale P, Faisant M, Guennoc B, Dupin M, Heyraud JD. Erythema dyschromicum perstans: report of a new case and critical review of the literature. J Dermatol. Nov 1998;25(11):747-53. [Medline].

  19. Convit J, Piquero-Martín J, Perez RM. Erythema dyschromicum perstans. Int J Dermatol. Apr 1989;28(3):168-9. [Medline].

  20. Epps RE. Case reports: selected dermatoses in children of color. J Drugs Dermatol. Jan 2007;6(1):78-82. [Medline].

  21. Gellin GA, Hilger R. Erythema dyschromicum perstans (Ashy dermatosis). Arch Dermatol. 1974;110:963.

  22. Gougerot MH. Lichen atypiques ou invisibles pigmentogenes reveles par des pigmentations. Bull Soc Fr Dermatol Syphiligr. 1935;42:792-4.

  23. Gougerot MH. Lichen atypiques ou invisibles pigmentogenes. Bull Soc Fr Dermatol Syphiligr. 1935;42:894-8.

  24. Henderson CD, Tschen JA, Schaefer DG. Simultaneously active lesions of vitiligo and erythema dyschromicum perstans. Arch Dermatol. Aug 1988;124(8):1258-60. [Medline].

  25. Lambert WC, Schwartz RA, Hamilton GB. Erythema dyschromicum perstans. Cutis. Jan 1986;37(1):42-4. [Medline].

  26. Miyagawa S, Komatsu M, Okuchi T, Shirai T, Sakamoto K. Erythema dyschromicum perstans. Immunopathologic studies. J Am Acad Dermatol. May 1989;20(5 Pt 2):882-6. [Medline].

  27. Paradisi M, Mostaccioli S, Celano G, Angelo C, Ruatti P, Ferranti G, et al. [Erythema dischromicum perstans (ashy dermatosis). Report of two cases]. Pathologica. Sep-Oct 1993;85(1099):533-41. [Medline].

  28. Piquero-Martín J, Pérez-Alfonzo R, Abrusci V, Briceño L, Gross A, Mosca W, et al. Clinical trial with clofazimine for treating erythema dyschromicum perstans. Evaluation of cell-mediated immunity. Int J Dermatol. Apr 1989;28(3):198-200. [Medline].

  29. Ramirez C. The ashy dermatosis (erythema dyschromicum perstans): epidemiological study and report of 139 cases. Cutis. 1967;3:244-7.

  30. Ramirez O, Lopez Lino DG. [Current status of ashy dermatosis. Synonym--erythema dyschromicum perstans]. Med Cutan Ibero Lat Am. 1984;12(1):11-8. [Medline].

  31. Samos-Zielinksa J, Lancucki J. Chronic toxic erythemo-macular skin hyperpigmentation (erythema dyschromicum perstans. Przegl Dermatol. 1978;66:385-91.

  32. Schwartz RA. Erythema dyschromicum perstans: the continuing enigma of Cinderella or ashy dermatosis. Int J Dermatol. Mar 2004;43(3):230-2. [Medline].

  33. Sebbag N, Lacour JP. [Erythema dyschromicum perstans]. Ann Dermatol Venereol. Jan 2006;133(1):79-82. [Medline].

  34. Soter NA, Wand C, Freeman RG. Ultrastructural pathology of erythema dyschromicum perstans. J Invest Dermatol. Feb 1969;52(2):155-62. [Medline].

  35. Tschen JA, Tschen EA, McGavran MH. Erythema dyschromicum perstans. J Am Acad Dermatol. Apr 1980;2(4):295-302. [Medline].

  36. Zenorola P, Bisceglia M, Lomuto M. Ashy dermatosis associated with cobalt allergy. Contact Dermatitis. Jul 1994;31(1):53-4. [Medline].

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Further Reading

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Keywords

EDP, ashy dermatosis, dermatosis cenicienta, erythema chronicum figuratum melanodermicum, lichen planus

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Contributor Information and Disclosures

Author

Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of Preventive Medicine and Community Health, UMDNJ-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.

Medical Editor

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.

Pharmacy Editor

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.

Managing Editor

Jeffrey P Callen, MD, Professor of Medicine, 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 Honoraria Consulting; Centocor Honoraria Consulting

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine 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.

 
 
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