Inflammatory linear verrucous epidermal nevus

Alternate names

ILVEN; Linear verrucose epidermal nevus; Verrucous epidermal nevus; Verrucous nevus; Inflammatory linear verrucous epidermal naevus; Inflammatory linear verrucous epidermal nevus

Definition

Inflammatory linear verrucous epidermal nevus (ILVEN) is a type of skin overgrowth, called epidermal nevus.

Genetics

ILVEN is caused by a genetic change that occurs after conception (somatic mutation).

Signs and symptoms

• 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.
• It usually occurs alone; however, rarely ILVEN can be associated with other symptoms as part of an epidermal nevus syndrome.
• Rarely, ILVEN can become cancerous (for example, basal cell carcinoma or squamous cell carcinoma).

Diagnosis

• A diagnosis of ILVEN syndrome should be considered in patients with extensive verrucous, pruritic epidermal nevi, and/or systemic abnormalities.
• A thorough mucocutaneous, neurologic, ophthalmic, and orthopedic examination is necessary with specific investigations depending on the involved system.
• A regular follow-up is necessary due to the risk of malignant transformation of the nevi and development of systemic manifestations..

Treatment

• 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.
• Recently, several patients have been treated with laser therapy with good results.

NIH genetic and rare disease info

• NIH info on Inflammatory linear verrucous epidermal nevus

Inflammatory linear verrucous epidermal nevus is a rare disease.

Inflammatory linear verrucous epidermal nevus Resources
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