What are the other Names for this Condition? (Also known as/Synonyms)

• Dermatitic Epidermal Nevus
• ILVEN (Inflammatory Linear Verrucous Epidermal Nevus)

What is Inflammatory Linear Verrucous Epidermal Nevus? (Definition/Background Information)

• Inflammatory Linear Verrucous Epidermal Nevus (ILVEN) is an extremely rare skin condition that results in the formation of itchy skin lesions due to epidermis (skin) overgrowth
• ILVEN is a slow-growing, benign skin tumor that typically involves one of the lower legs. In rare cases, it can involve both legs; however, it may also develop anywhere on the body
• This condition is characterized by skin lesions that form in a linear manner. The lesions appear red and inflamed causing a lot of irritation and discomfort. These symptoms are very similar to psoriasis and eczema, which often causes a confusion or may lead to a misdiagnosis
• The cause of ILVEN is currently unknown. It arises due to a defect in the ectoderm during fetal development. The ectoderm is the germ layer that gives rise to the epidermis and skin
• The ectoderm is commonly associated with other birth defects, especially defects of the bones, eyes, and brain. This is due to the fact that other body structures also form from ectoderm
• In rare cases, the ILVEN skin lesions may become malignant and cancerous. Open lesions are also susceptible to bacterial infections, which can give rise to other health complications
• A treatment of Inflammatory Linear Verrucous Epidermal Nevus may include surgical excision of the lesions and the use of topical creams to ease itchiness. The prognosis of ILVEN is good, if appropriate treatment is provided in a timely manner

Who gets Inflammatory Linear Verrucous Epidermal Nevus? (Age and Sex Distribution)

• Inflammatory Linear Verrucous Epidermal Nevus typically occurs in the first 6-months of life. It can also occur in children after the age of five, although this is a rare occurrence. ILVEN is very rarely observed during adulthood
• Females are affected much more by ILVEN, than males (female-male ratio is 4:1)
• This skin disorder can occur worldwide; there is no ethnic or racial preference

What are the Risk Factors for Inflammatory Linear Verrucous Epidermal Nevus? (Predisposing Factors)

Risk factors of Inflammatory Linear Verrucous Epidermal Nevus (ILVEN) include:

• Rarely, the presence of epidermal nevus syndrome: Epidermal nevus syndrome is a congenital defect of the ectodermal layer. ILVEN is linked to this syndrome, because epidermal nevus syndrome develops due to defects in the ectodermal layer, where the epidermis originates
• These birth defects, especially those associated with the bones, eyes, and brain, can rarely be seen with ILVEN as part of epidermal nevus syndrome

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider. 

What are the Causes of Inflammatory Linear Verrucous Epidermal Nevus? (Etiology)

• The exact cause of development of Inflammatory Linear Verrucous Epidermal Nevus (ILVEN) is presently unknown. They are thought to occur spontaneously
• Some research has shown that ILVEN is caused by genetic mutations. However, cases where these specific genetic mutations have occurred are rare
• Currently, research is underway to improve our understanding of ILVEN

What are the Signs and Symptoms of Inflammatory Linear Verrucous Epidermal Nevus?

Common signs and symptoms of Inflammatory Linear Verrucous Epidermal Nevus (ILVEN) include:

• Pigmented skin lesions
• Skin lesions that appear to be clusters of warts
• Skin lesions that are present in lines (linear in appearance)
• Itchiness or redness at the site of the skin lesions
• Resemblance to common skin conditions, such as psoriasis and eczema

The skin lesions are most commonly present on the left leg. The reason for this is unknown. Although ILVEN typically occurs in the lower legs, it can affect any part of the body. 

How is Inflammatory Linear Verrucous Epidermal Nevus Diagnosed?

Inflammatory Linear Verrucous Epidermal Nevus (ILVEN) is diagnosed using a variety tests and exams that include:

• A thorough physical examination and a complete medical history, which are crucial for a proper diagnosis of ILVEN
• Dermoscopy: A dermoscopy is a diagnostic tool in which a dermatologist examines the affected skin, using a special magnified lens
• A tissue biopsy of the lesion: A tissue biopsy is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy under a microscope and puts together the clinical findings. This allows the pathologist to arrive at a definitive diagnosis. Examination of the biopsy under a microscope by a pathologist is considered to be the gold standard, in arriving at a conclusive diagnosis
• Sometimes, during the biopsy study, the pathologist may perform additional (special) studies, which may include immunohistochemical stains, histochemical stains, and molecular testing
• Differential diagnosis, to eliminate other lesion types are often considered, before arriving at a definitive diagnosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis. 

What are the possible Complications of Inflammatory Linear Verrucous Epidermal Nevus?

Individuals with Inflammatory Linear Verrucous Epidermal Nevus (ILVEN) may develop certain complications, such as:

• Some lesions are potentially pre-malignant and can become cancerous, if left untreated. About one in five individuals may have malignant cancers developing from these skin lesions, usually later in life
• Skin infections have also been noted in patients with ILVEN. Such infections are due to bacteria infecting skin ulcerations
• Complications may also arise during treatment. Scarring of skin is common, when patients with ILVEN receive treatment. This could lead to significant social and self-esteem issues

How is Inflammatory Linear Verrucous Epidermal Nevus Treated?

It is difficult to treat Inflammatory Linear Verrucous Epidermal Nevus (ILVEN) skin condition. Some of the treatment options may include:

• Medical professionals typically prescribe topical steroid creams, which work to decrease the inflammation and relieve itching
• Topical calcipotriol cream may also be prescribed, which helps with skin dryness. This cream should be used with care, since the medication can have toxic side effects
• Topical retinoids, such as retinoic acid and oral retinoids (isotretinoin), are sometimes used in ILVEN
• If the skin lesions caused by ILVEN are small, then they may be surgically excised. Larger skin lesions are typically treated using CO2 (carbon dioxide) laser surgery, pulsed dye laser, or others

A team of healthcare providers is usually necessary to properly treat the condition, if ILVEN is part of a syndrome. Since, other defects also typically exist with the syndrome, in such cases. This team should include neurologists, ophthalmologists, orthopedic surgeons, dermatologists, and social workers.

How can Inflammatory Linear Verrucous Epidermal Nevus be Prevented?

• The prevention of Inflammatory Linear Verrucous Epidermal Nevus (ILVEN) is difficult because the cause of the condition is unknown. Currently, there are no known methods to prevent ILVEN occurrence
• An early diagnosis and close monitoring of the patient’s condition with treatment of the skin lesions is important

What is the Prognosis of Inflammatory Linear Verrucous Epidermal Nevus? (Outcomes/Resolutions)

• The prognosis of Inflammatory Linear Verrucous Epidermal Nevus (ILVEN) is generally good, as long as regular follow-ups with medical professionals are observed. Early recognition and prompt treatment will help in having optimal outcomes
• If skin cancer develops from malignant transformation of ILVEN, good prognosis may be ensured with early detection and suitable treatment
• If such skin cancers are not detected early, then the prognosis may be poor if metastasis to different parts of the body takes place

Additional and Relevant Useful Information for Inflammatory Linear Verrucous Epidermal Nevus:

An epidermal nevus is a benign, yellowish-brown, wart-like skin lesion, which is commonly present at birth, or develops during childhood. The skin lesions may appear as plaques or papules, and are usually present in the trunk or extremities. 

The following article link will help you understand epidermal nevus:

https://www.dovemed.com/diseases-conditions/epidermal-nevus-en/