Erythrokeratodermia Figurata Variabilis

Erythrokeratodermia figurata variabilis (EFV) is a rare skin condition that can affect individuals of all ages. It’s important to understand the types, causes, symptoms, diagnostic tests, treatments, and drugs associated with EFV. In this simplified guide, we will break down complex medical jargon into plain English to enhance readability and accessibility.

Types of Erythrokeratodermia Figurata Variabilis:

EFV is generally categorized into two types:

  1. Classic Type: This type involves patches of reddish skin with thickened and scaly areas. These patches often have irregular shapes, resembling figures or patterns, hence the name “figurata.”
  2. Progressive Symmetrical Erythrokeratodermia (PSEK): In PSEK, the reddish patches are symmetrical and usually appear on both sides of the body. Unlike the classic type, PSEK doesn’t typically form distinct figures or patterns.

Causes of Erythrokeratodermia Figurata Variabilis:

The exact cause of EFV remains unclear, but it is believed to be a genetic disorder. Mutations in certain genes are thought to play a role in its development. EFV is not contagious and cannot be acquired from contact with an affected individual.

EFV is primarily caused by changes in a person’s genes (mutations). Here are some factors that can contribute to EFV:

  1. Genetic Mutations: Inherited genetic mutations play a significant role in EFV. These mutations affect the way the skin cells grow and develop.
  2. Family History: If someone in your family has EFV, you may be more likely to develop it.
  3. Spontaneous Mutations: In some cases, EFV can occur without any family history of the condition due to spontaneous gene mutations.

Symptoms of Erythrokeratodermia Figurata Variabilis:

Symptoms of EFV can vary from person to person, but common signs include:

  1. Reddish Patches: The skin often develops reddish patches that may be itchy or uncomfortable.
  2. Scaly Skin: These patches can become thickened and scaly, which can cause discomfort or pain.
  3. Change in Skin Pattern: In the classic type, the skin can develop distinct patterns or figures, while in PSEK, the patches are usually symmetrical.
  4. Varying Intensity: The severity of symptoms can vary over time, with flare-ups and periods of remission.
  5. No Other Systemic Symptoms: EFV typically only affects the skin and doesn’t cause other systemic health problems.

Diagnostic Tests for Erythrokeratodermia Figurata Variabilis:

Diagnosing EFV usually involves:

  1. Clinical Examination: A dermatologist will examine your skin and ask about your symptoms and family history.
  2. Genetic Testing: A genetic test may be performed to identify specific mutations associated with EFV.
  3. Skin Biopsy: In some cases, a small skin sample may be taken for examination under a microscope to confirm the diagnosis.

Treatments for Erythrokeratodermia Figurata Variabilis:

While there is no cure for EFV, various treatments can help manage symptoms:

  1. Emollients: Using moisturizers can help keep the skin hydrated and reduce scaling.
  2. Topical Steroids: These anti-inflammatory creams can reduce redness and itching during flare-ups.
  3. Oral Medications: In severe cases, oral medications like retinoids or antihistamines may be prescribed to control symptoms.
  4. Phototherapy: Some individuals find relief with ultraviolet (UV) light therapy.
  5. Laser Therapy: Laser treatments may be considered for specific skin lesions.
  6. Pain Management: Over-the-counter pain relievers can help manage discomfort.

Drugs Used in Erythrokeratodermia Figurata Variabilis:

  1. Topical Steroids: Common topical steroids include hydrocortisone and betamethasone.
  2. Oral Retinoids: Isotretinoin is a retinoid medication used in severe cases.
  3. Antihistamines: These can help relieve itching, with options like cetirizine and loratadine.
  4. Emollients: Plain moisturizers without added fragrances or irritants are often recommended.
  5. Pain Relievers: Over-the-counter pain relievers like ibuprofen or acetaminophen can help manage discomfort.

In conclusion, Erythrokeratodermia Figurata Variabilis is a rare skin condition that primarily affects the skin’s appearance and texture. It is believed to have a genetic basis and can vary in presentation. While there is no cure, various treatments and medications are available to manage symptoms and improve the quality of life for those affected by EFV. If you suspect you have EFV, consult with a dermatologist for a proper diagnosis and personalized treatment plan.

 

Disclaimer: Each person’s journey is unique, treatment plan, life style, food habit, hormonal condition, immune system, chronic disease condition, previous medical  history is also unique. So always seek the best advice from a qualified medical professional or health care provider before trying any treatments to ensure to find out the best plan for you. This guide is for general information and educational purposes only. If you or someone are suffering from this disease condition bookmark this website or share with someone who might find it useful! Boost your knowledge and stay ahead in your health journey. Thank you for giving your valuable time to read the article.

  1. https://medlineplus.gov/skinconditions.html
  2. https://www.aad.org/about/burden-of-skin-disease
  3. https://www.usa.gov/federal-agencies/national-institute-of-arthritis-musculoskeletal-and-skin-diseases
  4. https://www.cdc.gov/niosh/topics/skin/default.html
  5. https://www.skincancer.org/
  6. https://illnesshacker.com/
  7. https://endinglines.com/
  8. https://www.jaad.org/
  9. https://www.psoriasis.org/about-psoriasis/
  10. https://books.google.com/books?
  11. https://www.niams.nih.gov/health-topics/skin-diseases
  12. https://cms.centerwatch.com/directories/1067-fda-approved-drugs/topic/292-skin-infections-disorders
  13. https://www.fda.gov/files/drugs/published/Acute-Bacterial-Skin-and-Skin-Structure-Infections—Developing-Drugs-for-Treatment.pdf
  14. https://dermnetnz.org/topics
  15. https://www.aaaai.org/conditions-treatments/allergies/skin-allergy
  16. https://www.sciencedirect.com/topics/medicine-and-dentistry/occupational-skin-disease
  17. https://aafa.org/allergies/allergy-symptoms/skin-allergies/
  18. https://www.nibib.nih.gov/
  19. https://rxharun.com/rxharun/rxharun/article-types/skin-care-beauty/skin-diseases-types-symptoms-treatment/
  20. https://www.nei.nih.gov/
  21. https://en.wikipedia.org/wiki/List_of_skin_conditions
  22. https://en.wikipedia.org/?title=List_of_skin_diseases&redirect=no
  23. https://en.wikipedia.org/wiki/Skin_condition
  24. https://oxfordtreatment.com/
  25. https://www.nidcd.nih.gov/health/
  26. https://consumer.ftc.gov/articles/w
  27. https://www.nccih.nih.gov/health
  28. https://catalog.ninds.nih.gov/
  29. https://www.aarda.org/diseaselist/
  30. https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets
  31. https://www.nibib.nih.gov/
  32. https://www.nia.nih.gov/health/topics
  33. https://www.nichd.nih.gov/
  34. https://www.nimh.nih.gov/health/topics
  35. https://www.nichd.nih.gov/
  36. https://www.niehs.nih.gov
  37. https://www.nimhd.nih.gov/
  38. https://www.nhlbi.nih.gov/health-topics
  39. https://obssr.od.nih.gov/
  40. https://www.nichd.nih.gov/health/topics
  41. https://rarediseases.info.nih.gov/diseases
  42. https://beta.rarediseases.info.nih.gov/diseases
  43. https://orwh.od.nih.gov/

References