Necrolytic Acral Erythema
- Author: Katherine Z Holcomb, MD, MPH; Chief Editor: Dirk M Elston, MD more...
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]
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.
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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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].
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].
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].
Manzur A, Siddiqui AH. Necrolytic acral erythema: successful treatment with topical tacrolimus ointment. Int J Dermatol. Oct 2008;47(10):1073-5. [Medline].

