Necrolytic Acral Erythema Workup

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

Laboratory Studies

Hepatitis A, B, and C serologies: These are the most important tests because necrolytic acral erythema (NAE) has a high association with hepatitis C.

Assess liver function, including aspartate aminotransferase, alanine aminotransferase, bilirubin, protein, albumin, and alkaline phosphatase values. Some authors believe the lesions are most active during the active stage of hepatitis. The severity of the lesions does not correlate with the severity of the hepatitis.

Serum zinc, serum glucagon, and serum amino acid levels: These values have been reduced in some reported cases. Zinc deficiency associated with necrolytic acral erythema was noted by Najarian et al.[15]

Serum glucose and hemoglobin A1C levels: Consider performing these tests because necrolytic acral erythema may be more common in persons with diabetes and hepatitis C.

Najarian et al has associated hypozincemia and hyperzincuria with necrolytic acral erythema.[16]

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Imaging Studies

CT scanning of the liver and pancreas can be considered as an ancillary assessment. In some reported cases, the pancreas was imaged, most likely because some believe necrolytic acral erythema is a subset of necrolytic migratory erythema, a condition indicative of pancreatic glucagonoma. No pancreatic masses were found any of the patients imaged.

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Procedures

Consider skin biopsy to substantiate the diagnosis of necrolytic acral erythema. Consider liver biopsy to assess the status of hepatitis and before the institution of hepatitis C treatment.

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Histologic Findings

Necrolytic acral erythema is perhaps best defined clinically rather than histologically. Bentley et al noted a case of necrolytic acral erythema without classic histology findings, which responded to oral zinc therapy.[17]

In 2004, Abdallah et al[12] noted the following histopathological features:

  • Early stage - Nummular dermatitis–like moderate and regular acanthosis, as follows:
    • Variable spongiosis
    • Inflammatory infiltrate
    • Pigment incontinence
  • Fully evolved stage - Psoriasiform epidermal hyperplasia, as follows:
    • Marked papillomatosis
    • Parakeratosis
    • Focal hypergranulosis
    • Pigment incontinence
    • Occasional subcorneal pustules
    • Epidermal pallor
    • Vascular ectasia
    • Infiltrate of inflammatory cells in the papillary dermis
    • Necrotic keratinocytes - Sometimes become confluent in the upper epidermis, sometimes tracking along the acrosyringium
  • Late stage samples - Minimal-to-moderate acanthosis, as follows:
    • Minimal-to-moderate inflammatory cell infiltrate
    • Pigment incontinence

Others have noted epidermal necrosis with or without blister formation; vacuolar degeneration of the basal layer; and psoriatic changes with acanthosis, papillomatosis, hyperkeratosis, and parakeratosis. All of these are commonly found in persons in deficient nutritional states. Dermal findings have included a nonspecific superficial perivascular and interstitial lymphocytic dermatitis.

El-Ghandour et al[9] noted that electron microscope examination of patients with necrolytic acral erythema demonstrated clumped tonofilaments in the keratinocytes, yet hepatitis C virus RNA could not be detected in the plaques of necrolytic acral erythema.

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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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