Rare Neurology News

Disease Profile

Inflammatory linear verrucous epidermal nevus

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

-

ICD-10

Q82.5

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

ILVEN; Linear verrucose epidermal nevus; Verrucous epidermal nevus;

Categories

Rare Cancers; Skin Diseases

Summary

Inflammatory linear verrucous epidermal nevus (ILVEN) is a type of skin overgrowth, called epidermal nevus. It is characterized by skin colored, brown, or reddish, wart-like papules (nevi). The nevi join to form patches or plaques that often follow a pattern on the skin known as the "lines of Blaschko". The affected areas of the skin may be red, itchy, and inflamed. ILVEN typically presents from birth to early childhood and often is limited to one side of the lower half of the body. It affects females more often than males.[1][2][3] It usually occurs alone; however, rarely ILVEN can be associated with other symptoms as part of an epidermal nevus syndrome.[2] Rarely, ILVEN can become cancerous (for example, basal cell carcinoma or squamous cell carcinoma).[4]

ILVEN is caused by a genetic change that occurs after conception (somatic mutation). How this alteration leads to the signs and symptoms of ILVEN is not well understood.[3][4] There is no one treatment or cure for ILVEN. Products used to soften the skin (emollients), moisturizers, and topical steroids may be utilized to relieve symptoms of dryness and itching.[1] Recently, several patients have been treated with laser therapy with good results.[5]

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Learn more

    These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

    Where to Start

    • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.

      In-Depth Information

      • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
      • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
      • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
      • PubMed is a searchable database of medical literature and lists journal articles that discuss Inflammatory linear verrucous epidermal nevus. Click on the link to view a sample search on this topic.

        Selected Full-Text Journal Articles

          References

          1. Vanessa Ngan. Inflammatory linear verrucous epidermal nevus. DermNet NZ. 2003; https://www.dermnetnz.org/lesions/ilven.html.
          2. Teresa S Wright. Epidermal nevus and epidermal nevus syndrome. UpToDate. July 2016; https://www.uptodate.com/contents/epidermal-nevus-and-epidermal-nevus-syndrome.
          3. Robert A Schwartz. Epidermal Nevus Syndrome Clinical Presentation. Medscape. Jun 6, 2016; https://emedicine.medscape.com/article/1117506-clinical#showall.
          4. C. Anand Kumar, Garima Yeluri, Namita Raghav. Inflammatory linear verrucous epidermal nevus syndrome with its polymorphic presentation A rare case report. Contemp Clin Dent.. Jan-Mar 2012; v.3(1):https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3341748/.
          5. Osman MA & Kassab AN. Carbon dioxide laser versus erbium:YAG laser in treatment of epidermal verrucous nevus: a comparative randomized clinical study. J Dermatolog Treat. November 13, 2016; 1-6. https://www.ncbi.nlm.nih.gov/pubmed/27796132.
          6. S. M. Ravi Prakash, Swati Gupta, Nagaraju Kamarthi, Sumit Goel. Inflammatory linear verrucous epidermal nevus and regional odontodysplasia: A rare sorority. Indian J Dent. Oct-Dec 2015; 6(4):203-206. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4691991/.