Inflammatory Linear Verrucous Epidermal Nevus (ILVEN)

Inflammatory Linear Verrucous Epidermal Nevus (ILVEN) is a rare skin disease that appears as a kind of overgrowth on the skin. ILVEN is named by the color of the affected skin, as reddish or brown, and by the papules (nevi).

The nevi are characterized as scaly plaques and patches that follow distinct lines of migration in the skin known as “the lines of Blaschko.” When the skin is affected by ILVEN, the site feels itchy or inflamed. This disorder is mostly found on the lower limbs of the body but may affect other parts as well.

ILVEN mostly affects newborn babies. It is rare for children older than 5 years to be affected. In exceptional cases, adults are also affected. Females are more prone to this condition than men. This skin disorder occurs all over the world irrespective of race or region.

Causes of ILVEN

The reasons for this condition are not known as of now. It is known to arise spontaneously, but some research points to the cause of ILVEN as a mutation. It is supposed that the mutation responsible is transmitted in an autosomal dominant manner. Currently, such cases are rare. Studies are on to better understand the cause of ILVEN.

Symptoms of ILVEN

The major symptoms of ILVEN include the following:

  • Skin lesions present as a birthmark
  • Pigmented skin lesions
  • Skin lesions that occur in a linear fashion
  • Frequent itchiness over the lesions
  • Lesions resembling common skin conditions such as eczema and psoriasis

Diagnosing ILVEN

A complete medical history and physical examination is required to diagnose ILVEN.

Tissue Examination: A sample of affected tissue is excised and sent for pathological examination. In the laboratory, the biopsy is examined under the microscope by the pathologist. This microscopic examination is the gold standard in determining the diagnosis. The histopathologic examination may also include additional tests such as histochemical stains, molecular studies, and immunohistochemical stain testing.

Dermoscopy: The examination of the affected skin by the dermatologist with the help of a magnifier lens is known as dermoscopy. It is important to eliminate other types of skin tumors which may have similar symptoms and appearance. These must be ruled out by careful examination.  

ILVEN Complications

Person affected with ILVEN may have other complications such as skin cancer, arising from untreated lesions. Recent research has shown that one in every five persons with skin lesions develops malignant cancers.

Patients with ILVEN may also develop serious bacterial skin infections. These bacteria largely gain entry through the ulceration in the skin.

Other complications may occur during the treatment period. These include scars which may adversely impact one’s social status and acceptance, and reduce the feeling of self-esteem.

Treatment for ILVEN

Medically, it is difficult to treat ILVEN. The usual medical therapies include:

  • Topical steroid creams which help to reduce the level of itching and inflammation
  • Topical calcipotriol helps to alleviate skin dryness, but has a higher level of toxicity

Surgical treatment includes surgical excision of small lesions, or carbon dioxide laser ablation of larger lesions. A team approach is advisable including a dermatologist, ophthalmologist, neurologist, and orthopedic surgeons.

Risk Factors for ILVEN

The risk factors predicting the development of ILVEN include:

Epidermal Nevus Syndrome (ENS): This is a congenital disorder of the ectodermal layer of skin which leads to abnormal growth of the epidermis.

Birth defects: Birth defects play a vital role in causing the ILVEN syndrome. People with ILVEN have a high incidence of congenital anomalies of the eyes, brain, and bone.

The presence of a risk factor alone does not always predict the development of ILVEN, but it does increase the risk of the condition. Even an individual without any risk factor may develop ILVEN. However, if any risk factors are present, it is wise to consult a healthcare provider and rule out the condition and work out a system to detect it if it develops.

Reviewed by Liji Thomas, MD.

References

Further Reading

Last Updated: Nov 6, 2017

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