Necrolytic acral erythemaL99.8

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 19.06.2022

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Synonym(s)

Acral necrolytic erythema; Necrolytic acral erythema

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HistoryThis section has been translated automatically.

el Darouti and Abu el Ela 1996. The first description of the disease was in Egypt. Later cases are in the Middle East, the USA and India.

DefinitionThis section has been translated automatically.

Disease pattern belonging to thenecrolytic erythemas(erythema necrolyticum migrans, acrodermatitis enteropathica) (there is an overlap with acrodermatitis enteropathica due to the frequently detectable zinc deficiency) that is predominantly associated with hepatitis C infection (Dabas G et al. 2018).

Occurrence/EpidemiologyThis section has been translated automatically.

The cases published to date relate quite predominantly to patients from Asia and Africa.

EtiopathogenesisThis section has been translated automatically.

The skin lesions usually occur in patients infected with hepatitis C virus (HCV). Cases of NAE have also been reported in patients without HCV infection. Hepatic dysfunction leading to metabolic changes such as hypoalbuminemia, hypoaminoacidemia, hyperglucagonemia, and transient zinc deficiency appear to be etiogenetically significant.

ManifestationThis section has been translated automatically.

Mostly with Pat. in the third to fifth decade of life

LocalizationThis section has been translated automatically.

Exclusively acral on the back of the hand and the back of the foot; palms of the hands and soles of the feet are left out.

Clinical featuresThis section has been translated automatically.

Burning or itchy, slowly spreading, rich red plaques with adherent, coarse lamellar, brown or brown-black scaling, sometimes also blistering with later necrosis formation (Geria AN et al. 2009).

LaboratoryThis section has been translated automatically.

HCV serology, zinc serum levels

HistologyThis section has been translated automatically.

Psoriasiform aspect with strong dermal neutrophilic inflammation, parakeratosis, hyperkeratosis, infiltration of the epidermis by neutrophilic granulocytes.

TherapyThis section has been translated automatically.

Treatment of present hepatitis C.

Oral zinc therapy is the most effective treatment for NAE, and is given in most cases regardless of HCV status or serum zinc levels.

Local therapy: Glucocorticoid creams.

Note(s)This section has been translated automatically.

Recently published cases without associated hepatitis C infection also suggest that other etiological relationships are possible (Srisuwanwattana P et al. 2017). Some authors doubt the entity of the clinical picture and suspect a clinical variant of the erythema necrolyticum migrans (necrolytic migratory erythema) (Nofal AA et al. 2005).

LiteratureThis section has been translated automatically.

  1. Dabas G et al. (2018) Necrolytic acral erythema leading to diagnosis of chronic hepatitis C. Digestive and liver disease: official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver 50: 854.
    Geria AN et al. (2009) Necrolytic acral erythema: a review of the literature. Cutis 83: 309-314.
  2. Inamadar AC et al (2020) Necrolytic acral erythema: current insights. Clin Cosmet Investig Dermatol 13:275-281.

  3. Nofal AA et al (2005) Necrolytic acral erythema: A Variant of Necrolytic Migratory Erythema or a Distinct Entity? Int J Dermatol 44: 916-921
  4. Srisuwanwattana P et al (2017) Necrolytic acral erythema in seronegative hepatitis C. Case reports in dermatology 9:69-73.

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Last updated on: 19.06.2022