Necrolytic Acral Erythema 

  • Author: Katherine Z Holcomb, MD, MPH; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Aug 2, 2011
 

Background

Necrolytic acral erythema (NAE) was first described in 1996 by physicians in Egypt, M. El Darouti and M. Abu El Ela.[1] Reports have continued to link hepatitis C with NAE.[2] Necrolytic acral erythema manifests as well-circumscribed, dusky erythematous plaques with adherent scale. While the plaques are psoriasiform, they do not manifest an Auspitz sign as would be seen with psoriasis. Patients with active necrolytic acral erythema report burning or pruritus. It is limited to an acral distribution and, in most cases, is associated with hepatitis C infection.[3, 4] Several cases of necrolytic acral erythema have occurred in patients without hepatitis C.[5] This suggests that necrolytic acral erythema might be a result of zinc dysregulation, rather than a result of hepatitis C infection itself.

Authors debate whether necrolytic acral erythema is a distinct entity or a subtype of necrolytic migratory erythema. However, the distinct appearance and usual coincidence with hepatitis C infections suggests that it is a unique entity. It has been speculated that viral load and viral genotype might play a role in NAE.[6]

Another debate has arisen regarding cases of NAE that are seronegative for hepatitis C and whether these cases constitute a distinct and separate clinical subset of NAE.[7, 8]

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Pathophysiology

The pathophysiology of this condition is uncertain. Proposed theories for the cause of necrolytic acral erythema describe alterations in some metabolic factor, many of which are seen in other necrolytic erythemas, including necrolytic migratory erythema, pellagra, essential fatty acid and biotin deficiency, and acrodermatitis enteropathica. The hypothesized causes for the metabolic alteration include hypoalbuminemia, hypoaminoacidemia, low zinc level, increased glucagon, liver dysfunction, or diabetes. Only hepatitis C is universally present in all persons with necrolytic acral erythema.

An odd fact is that no cases of necrolytic acral erythema have been reported in Japan, which has a high seroprevalence rate of hepatitis C.

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Epidemiology

Frequency

United States

Only 2 cases of necrolytic acral erythema have been described in the United States to date, but the condition is likely more common than the case reports suggest.

International

Forty-two cases of necrolytic acral erythema have been described internationally, mostly in Egypt. El-Ghandour et al[9] in Egypt described a series of 23 patients (mean age, 41.7 ±11.5 y; male-to-female ratio, 10:13) with clinical features consistent with necrolytic acral erythema examined over a 3-year period. Most necrolytic acral erythema patients were adults (91.3%), and the skin lesions were predominantly chronic (78.3%), with the dorsa of the toes and/or feet affected in all cases.

Mortality/Morbidity

Necrolytic acral erythema has no known directly associated morbidly or mortality. Rather, the morbidity and mortality are related to the primary illness, hepatitis C.

Race

Most cases of necrolytic acral erythema have been reported in Egyptians. No cases have been reported in whites.

Sex

To date, no sex predisposition is reported for necrolytic acral erythema.

Age

The ages of the patients with necrolytic acral erythema have ranged from 11-60 years, but the onset typically occurs between 35-55 years.

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Contributor Information and Disclosures
Author

Katherine Z Holcomb, MD, MPH  Staff Physician, Department of Dermatology, Naval Hospital Camp Lejeune; Clinical Instructor, Department of Family Medicine, Naval Hospital Camp Lejeune

Katherine Z Holcomb, MD, MPH is a member of the following medical societies: American Academy of Dermatology, American Society for Dermatologic Surgery, and Women's Dermatologic Society

Disclosure: Nothing to disclose.

Coauthor(s)

Noah S Scheinfeld, MD, JD, FAAD  Assistant Clinical Professor, Department of Dermatology, Columbia University College of Physicians and Surgeons; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, and New York Eye and Ear Infirmary; Private Practice

Noah S Scheinfeld, MD, JD, FAAD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Optigenex Consulting fee Independent contractor

Specialty Editor Board

Timothy McCalmont, MD  Director, UCSF Dermatopathology Service, Professor of Clinical Pathology and Dermatology, Departments of Pathology and Dermatology, University of California at San Francisco; Editor-in-Chief, Journal of Cutaneous Pathology

Timothy McCalmont, MD is a member of the following medical societies: Alpha Omega Alpha, American Medical Association, American Society of Dermatopathology, California Medical Association, College of American Pathologists, and United States and Canadian Academy of Pathology

Disclosure: Apsara Consulting fee Independent contractor

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Camila K Janniger, MD  Clinical Professor of Dermatology, Clinical Associate Professor of Pediatrics, Chief of Pediatric Dermatology, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Camila K Janniger, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

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, Ackerman Academy of Dermatopathology, New York

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

Disclosure: Nothing to disclose.

References

  1. el Darouti M, Abu el Ela M. Necrolytic acral erythema: a cutaneous marker of viral hepatitis C. Int J Dermatol. Apr 1996;35(4):252-6. [Medline].

  2. Tabibian JH, Gerstenblith MR, Tedford RJ, Junkins-Hopkins JM, Abuav R. Necrolytic Acral Erythema as a Cutaneous Marker of Hepatitis C: Report of Two Cases and Review. Dig Dis Sci. May 26 2010;[Medline].

  3. Abdallah MA, Ghozzi MY, Monib HA, et al. Necrolytic acral erythema: a cutaneous sign of hepatitis C virus infection. J Am Acad Dermatol. Aug 2005;53(2):247-51. [Medline].

  4. Khanna VJ, Shieh S, Benjamin J, et al. Necrolytic acral erythema associated with hepatitis C: effective treatment with interferon alfa and zinc. Arch Dermatol. Jun 2000;136(6):755-7. [Medline].

  5. Wu YH, Tu ME, Lee CS, Lin YC. Necrolytic acral erythema without hepatitis C infection. J Cutan Pathol. Mar 2009;36(3):355-8. [Medline].

  6. El-Darouti MA, Mashaly HM, El-Nabarawy E, et al. Leukocytoclastic vasculitis and necrolytic acral erythema in patients with hepatitis C infection: do viral load and viral genotype play a role?. J Am Acad Dermatol. Aug 2010;63(2):259-65. [Medline].

  7. Panda S, Lahiri K. Seronegative necrolytic acral erythema: a distinct clinical subset?. Indian J Dermatol. Jul-Sep 2010;3:259-61. [Medline].

  8. Patel U, Loyd A, Patel R, Meehan S, Kundu R. Necrolytic acral erythema. Dermatol Online J. Nov 2010;11:15. [Medline].

  9. El-Ghandour TM, Sakr MA, El-Sebai H, El-Gammal TF, El-Sayed MH. Necrolytic acral erythema in Egyptian patients with hepatitis C virus infection. J Gastroenterol Hepatol. Jul 2006;21(7):1200-6. [Medline].

  10. Williams J. Necrolytic acral erythema. DermAtlas. Available at http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=178630456. Accessed January 25, 2007.

  11. Janjua SA. Necrolytic acral erythema. DermAtlas. Available at http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=178630456. Accessed January 25, 2007.

  12. Abdallah MA, Ghozzi MY, Monib HA, et al. Histological study of necrolytic acral erythema. J Ark Med Soc. Apr 2004;100(10):354-5. [Medline].

  13. Hivnor CM, Yan AC, Junkins-Hopkins JM, Honig PJ. Necrolytic acral erythema: response to combination therapy with interferon and ribavirin. J Am Acad Dermatol. May 2004;50(5 Suppl):S121-4. [Medline].

  14. Nofal AA, Nofal E, Attwa E, El-Assar O, Assaf M. Necrolytic acral erythema: a variant of necrolytic migratory erythema or a distinct entity?. Int J Dermatol. Nov 2005;44(11):916-21. [Medline].

  15. Najarian DJ, Lefkowitz I, Balfour E, Pappert AS, Rao BK. Zinc deficiency associated with necrolytic acral erythema. J Am Acad Dermatol. Nov 2006;55(5 Suppl):S108-10. [Medline].

  16. Najarian DJ, Najarian JS, Rao BK, Pappert AS. Hypozincemia and hyperzincuria associated with necrolytic acral erythema. Int J Dermatol. Jul 2008;47(7):709-11. [Medline].

  17. Bentley D, Andea A, Holzer A, Elewski B. Lack of classic histology should not prevent diagnosis of necrolytic acral erythema. J Am Acad Dermatol. Mar 2009;60(3):504-7. [Medline].

  18. Abdallah MA, Hull C, Horn TD. Necrolytic acral erythema: a patient from the United States successfully treated with oral zinc. Arch Dermatol. Jan 2005;141(1):85-7. [Medline].

  19. de Carvalho Fantini B, Matsumoto FY, Arnone M, Sotto MN, Junior WB. Necrolytic acral erythema successfully treated with oral zinc. Int J Dermatol. Aug 2008;47(8):872-3. [Medline].

  20. Manzur A, Siddiqui AH. Necrolytic acral erythema: successful treatment with topical tacrolimus ointment. Int J Dermatol. Oct 2008;47(10):1073-5. [Medline].

 
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Plaque of necrolytic acral erythema on the ankle of a male.
 
 
 
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