ICD-11 Codes
for EA20 - Necrolytic acral erythema

Teodor Jurukovski January 1, 1970

Fact checked by: Teodor Jurukovski

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Understanding Necrolytic Acral Erythema (ICD-11 Code EA20)

Necrolytic acral erythema, identified by the ICD-11 code EA20, is a rare and distinct dermatological condition primarily associated with chronic hepatitis C virus (HCV) infection. While most commonly linked to HCV, it can also manifest due to zinc deficiency. This condition characteristically affects the extremities, particularly the hands and feet, presenting as painful, erythematous, and eroded skin patches, which may sometimes develop into fluid-filled blisters. It is often recognised as a significant cutaneous marker for underlying hepatitis C infection, sometimes being the first indication of the disease.

Synonyms and Alternative Terms for Necrolytic Acral Erythema

The condition officially classified under ICD-11 code EA20 is known by several terms. The primary official name is Necrolytic acral erythema. Common clinical synonyms and patient-friendly descriptions include zinc-responsive necrolytic acral erythema and zinc-responsive seronegative necrolytic acral erythema. When documenting or searching for information, terms such as 'skin rash associated with hepatitis C' or 'acral dermatosis EA20' may also be encountered.

Common Symptoms and Presentation of Necrolytic Acral Erythema

Necrolytic acral erythema typically presents as a symmetrical rash affecting the skin of the toes and the tops of the feet. Lesions may also appear on the ankles, knees, legs, and less commonly on the hands, elbows, genitalia, and buttocks, though the palms, soles, and nails are usually spared. The skin lesions are often described as painful, though some individuals may experience itching or a burning sensation.

The presentation can vary through distinct stages:

• Initial Phase: Small papules and plaques appear, which are scaly with a dusky or eroded centre.
• Well-Developed Phase: These lesions coalesce to form larger, well-demarcated, hyperpigmented, thick plaques with adherent scales. Occasional pustules may be present.
• Late Phase: Plaques become thinner, more circumscribed, and psoriasiform, often with a well-defined dark red rim and hyperpigmentation.

In its acute form, lesions appear as red patches with margins of erosions or flaccid blisters. Chronic forms often present as hyperkeratotic, erythematous eruptions.

Causes and Risk Factors for Necrolytic Acral Erythema

The aetiopathogenesis of necrolytic acral erythema (EA20) is multifactorial. The most significant risk factor is chronic infection with the Hepatitis C Virus (HCV), with over 75% of affected individuals testing positive for the virus. The exact mechanism by which HCV contributes to this skin condition is not fully understood but may involve direct viral effects, immune responses, or metabolic disturbances linked to liver dysfunction. Low blood levels of albumin, glucagon, and amino acids are also implicated.

Zinc deficiency is another key factor, sometimes occurring in conjunction with other disorders such as coeliac disease or Crohn disease. While zinc deficiency can be a cause, oral zinc supplementation is often found to be effective even in patients without a diagnosed deficiency, suggesting a complex role for zinc in the condition's pathophysiology. Viral load and immunosuppression are not considered primary risk factors.

How Necrolytic Acral Erythema is Diagnosed

Diagnosing necrolytic acral erythema (ICD-11 code EA20) involves a comprehensive clinical evaluation and specific investigations. Suspicion arises from characteristic skin signs in patients with relevant risk factors.

Diagnostic approaches include:

• Clinical Examination: Assessing the typical acral distribution, appearance, and stages of the skin lesions.
• Skin Biopsy: Histological examination may reveal hyperkeratosis with parakeratosis, epidermal spongiosis, focal necrosis, and a superficial dermal inflammatory infiltrate, often similar to necrolytic migratory erythema.
• Blood Tests: Essential investigations include:
• Hepatitis C serology (crucial for identifying underlying infection).
• Liver function tests (LFTs) to assess liver health.
• Serum zinc levels (may be normal or low).
• Amino acid and albumin levels (can indicate metabolic disturbances).
• Hepatitis B and HIV serology (due to similar transmission routes).
• Imaging: Ultrasound studies may be performed, particularly in patients with HCV, to evaluate liver status.

Normal glucagon levels can help differentiate necrolytic acral erythema from necrolytic migratory erythema.

Treatment and Management Considerations for Necrolytic Acral Erythema

The management of necrolytic acral erythema (EA20) focuses on addressing both the underlying cause and the cutaneous manifestations. Effective treatment strategies often involve:

• Oral Zinc Supplementation: Administering oral zinc supplements, typically 220 mg twice daily, is highly effective in improving skin lesions, even in individuals without a confirmed zinc deficiency.
• Treatment of Underlying Cause: For patients with Hepatitis C virus (HCV) infection, antiviral therapies such as ribavirin and interferon-alpha have shown efficacy in improving the skin condition. Addressing malabsorption issues is also important if present.

Other treatments, including topical corticosteroids, calcineurin inhibitors (like tacrolimus), and phototherapy, have generally shown limited or mixed results and are not considered primary treatment modalities.

Documentation and Coding for Necrolytic Acral Erythema

Accurate documentation and coding are vital for managing patients with necrolytic acral erythema (ICD-11 code EA20). The official ICD-11 code for this condition is EA20. When documenting, healthcare providers should include details about the clinical presentation, diagnostic findings (such as HCV status, zinc levels, LFTs), and the treatment plan. This condition can serve as an important diagnostic clue for underlying hepatitis C or zinc deficiency, necessitating thorough investigation. For billing and reporting purposes, using the precise ICD-11 code EA20 ensures accurate classification of this specific skin disorder attributable to viral infection or nutritional deficiency.