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Necrolytic Acral Erythema Clinical Presentation

  • Author: Katherine Z Holcomb, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Jan 25, 2016
 

History

Patients with necrolytic acral erythema present with a 1-month to 2-year history of a rash on the dorsum of the feet, which may or may not also involve the hands. In many cases, it has been unresponsive to topical steroids. Itching can precede the development of the rash. Sometimes, purulent discharge occurs from the lesions of necrolytic acral erythema.[20]

Patients do not necessarily give a history of hepatitis C. Necrolytic acral erythema may be the presenting sign. Patients may report a burning sensation, especially when walking or standing. Sometimes, necrolytic acral erythema lesions are pruritic. The most common area of origination of the rash is the dorsal aspect of the great toe. It also occurs on the shins. In 43 of 44 reported cases, necrolytic acral erythema did not affect the palms or soles, the nail bed, nail plate, or mucous membranes.

Williams[21] reported a case of necrolytic acral erythema in an adolescent boy with a history of infection with hepatitis C virus; he had hepatic fibrosis, hypertension, and thrombocytopenia. The patient developed a pruritic eruption on his face, trunk, genitals, and extremities, and the eruption had a predilection for bony prominences. This patient did not respond to topical steroids, antihistamines, topical barrier repair creams, narrow-band UV-B therapy, or tar baths. A skin biopsy specimen demonstrated a nonspecific psoriasiform dermatitis consistent with necrolytic acral erythema. Hepatitis C virus RNA polymerase chain reaction studies showed 974,000 IU/mL (< 50) and hepatitis C virus RNA genotype 1b.

Janjua[22] noted a 45-year-old man who developed well-defined erythematous hyperpigmented keratotic pruritic plaques on the dorsa of his feet and the lateral aspect of his ankles; he was determined to have necrolytic acral erythema.

Necrolytic acral erythema after hepatitis B vaccination was noted in 2014.[23]

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Physical

Dusky, red erythematous plaques are present bilaterally on the dorsum of the feet and toes. These may extend around to the skin overlying the Achilles tendon and up the lower leg, as depicted in the image below.

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

A key finding of necrolytic acral erythema is that while the plaques have a psoriatic morphology, they do not show the psoriasis Auspitz sign (ie, if you pick at the scale, it does not bleed).[24] Active necrolytic acral erythema has different symptoms from psoriasis. The plaques of psoriasis itch, and the plaques of necrolytic acral erythema can express burning or pruritus. Usually lesions do not occur on the body just like psoriasis. Necrolytic acral erythema is an all-acral disease. Some necrolytic acral erythema patients have zinc dysregulation and not hepatitis C infection, showing the complexity of the disease.

The dorsum of the hands may or may not be involved. The lesions are clearly demarcated from uninvolved skin by a dark red border. The surface may be scaly, eroded, or velvety. Thick hyperkeratosis is sometimes present. Active lesions often include flaccid blisters. Edema may or may not be present.

Abdallah et al[25] describe the following stages of the lesions:

  1. Initial stage: Erythematous papules or plaque with scale are present and have a dusky or eroded center.
  2. Fully developed stage: A confluence of papules and plaques with sharply defined margins and adherent scale develops. Increased hyperpigmentation and decreased redness may be present. Lesions may be lichenified. Pustules also may occur at this stage.
  3. Late stage: Thinning of lesions occurs, with continued hyperpigmentation. Demarcation continues, followed by spontaneous relapse and remission.

As a general rule, necrolytic acral erythema does not affect the palms or soles, the nail bed, nail plate, or mucous membranes. Hivnor et al[26] reported a single case in which the plaques extended proximally to the thighs. This patient also had hyperkeratosis of the palms and soles and involvement of the face. While multiple biopsy specimens confirmed necrolytic acral erythema histologically, it is not clear whether these biopsy specimens were taken from the typical locations of necrolytic acral erythema or if the palms and soles were also included in the biopsy specimen.

Other disorders that can be seen in persons with hepatitis C should be considered as possible epiphenomena. These include the following:

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Causes

The cause is uncertain, but a metabolic alteration due to hepatocellular degeneration from hepatitis C infection is proposed. Many hypotheses describe deficiencies similar to those of the other necrolytic erythemas; histological features are similar among necrolytic acral erythema and other nutrient deficiencies.

el Darouti and Abu El Ela[1] suggested that the increased levels of glucagon seen in persons with liver disease allow for "potentiation" of arachidonic acid after trauma.

High glucagon levels alone may allow for greater arachidonic acid potentiation, which may induce inflammatory changes and necrosis in the epidermis.

Low amino acid levels may lead to epidermal protein depletion and necrolysis.

Low albumin levels have also been postulated as causative. Albumin sequesters fatty acids and helps regulate prostaglandin levels. High levels of prostaglandins as a result of low levels of albumin may induce inflammation.

Low zinc levels have also been proposed as a cause. Because albumin is the main carrier of zinc in plasma, these 2 may be interrelated.

Diabetic microangiopathy also may play a role; 4 of 5 necrolytic acral erythema patients described by Nofal et al[27] also had diabetes.

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Contributor Information and Disclosures
Author

Katherine Z Holcomb, MD, MPH Dermatologist, Lupo Center for Aesthetic and General Dermatology; Clinical Assistant Professor, Department of Derrmatolgy, Tulane University School of Medicine

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

Disclosure: Nothing to disclose.

Coauthor(s)

Noah S Scheinfeld, JD, MD, FAAD Assistant Clinical Professor, Department of Dermatology, Weil Cornell Medical College; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Assistant Attending Dermatologist, New York Presbyterian Hospital; Assistant Attending Dermatologist, Lenox Hill Hospital, North Shore-LIJ Health System; Private Practice

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

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Abbvie<br/>Received income in an amount equal to or greater than $250 from: Optigenex<br/>Received salary from Optigenex for employment.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

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

Disclosure: Nothing to disclose.

Camila K Janniger, MD Clinical Professor of Dermatology, Clinical Associate Professor of Pediatrics, Chief of Pediatric Dermatology, Rutgers New Jersey Medical School

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

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

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

Disclosure: Nothing to disclose.

Additional Contributors

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, United States and Canadian Academy of Pathology

Disclosure: Received consulting fee from Apsara for independent contractor.

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