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Erythrokeratoderma

Genetic disease with well-demarcated fixed erythematous plaques and hyperkeratotic areas on the skin.

Written by: Saygı Hospital Health Guide Editorial Board
Published:

This content is for general information; please consult your physician for diagnosis and treatment.

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Dermatoloji department. Book Appointment →

What is Erythrokeratoderma?

Erythrokeratoderma is a group of rare, well-defined genetic dermatoses with erythematous plaques accompanied by hyperkeratotic areas. There are two main forms: progressive symmetric erythrokeratoderma and erythrokeratoderma variabilis.

In the progressive symmetric form, slowly enlarging, symmetric, hyperkeratotic red plaques appear on the legs and arms. In the variabilis form, lesions change shape and location within hours or days; this form is associated with GJB3 and GJB4 mutations.

The disease begins in childhood and may persist throughout life. The psychological and cosmetic impact is significant. Treatment is symptomatic; keratolytics, retinoids, and emollients form the mainstay.

Symptoms

Sharply demarcated red plaques
Thick hyperkeratotic areas overlying plaques
Involvement of extensor surfaces of extremities
Lesions changing shape in the variabilis form
Itching and tightness sensation
Hyperkeratosis on palms and soles
Cosmetic discomfort

Risk Factors

Family history of erythrokeratoderma
GJB3, GJB4, or KDSR mutations
Childhood onset
Rare autosomal dominant inheritance
Cold, dry climate
Stress

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • If slowly enlarging red and scaly plaques appear in a child
  • If symmetric and painful plaques affect daily life
  • If palmoplantar thickening makes walking difficult
  • If diagnosis is uncertain and differentiation from psoriasis is needed
  • If genetic counseling is required

Treatment Methods

01
Topical retinoids and vitamin D analogues
02
Keratolytics (urea, salicylic acid)
03
Moisturizers and emollients
04
Low-dose systemic retinoids (selected cases)
05
Topical corticosteroids (short-term)
06
Genetic counseling and psychosocial support

Which Department to Visit?

You can visit our Dermatoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Let us help you

You can make an appointment with our specialists or contact us for your concerns.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.