ILVEN (Inflammatory Linear Verrucous Epidermal Nevus)

Key Points
Rare type of epidermal nevus which typically affects the legs
Caused by a defect in the ectoderm in the embryonic stage
Consists of inflamed, red lesions which are warty and form in a linear pattern, and are accompanied by itching, dryness, or scaling

Inflammatory linear verrucous epidermal nevus, or ILVEN, is a rare type of epidermal nevus which typically appears on one leg, and may extend to the buttocks and/or foot. The condition consists of inflamed, red lesions which are warty and form in a linear pattern. Additionally, the condition is accompanied by severe itching, and lesions may experience dryness or scaling.

ILVEN is caused by a defect in the ectoderm, the part of the embryo which forms the skin and neural tissues. While the condition may be congenital in some cases, it typically appears after birth, before the age of 5, and is slightly more common in females. In very rare cases, the defect may also cause internal disorders affecting the brain, eyes and skeleton.

Differential Diagnosis (Other conditions with similar appearance)Acrokeratosis verruciformis of Hopf
Lichen Simplex Chronicus
CHILD syndrome
Lichen Striatus
Epidermodysplasia verruciformis
Nevus comedonicus
Epidermolytic hyperkeratosis
Nevus Sebaceus
Erythrokeratodermia Variabilis
Porokeratosis
Keratosis Follicularis (Darier disease)
Proteus syndrome
Lichen Nitidus
Lichen Planus

Diagnosis
Key Points
Initial diagnosis based on the characteristic appearance of the affected area
Laboratory tests will be performed to confirm diagnosis and rule out other conditions

ILVEN is initially diagnosed based on the characteristic appearance of the affected area. Laboratory testing will be performed to confirm the diagnosis and to rule out similar appearing conditions.

Treatment
No cure for the condition exists
*Treatment consists of managing symptoms

No cure for ILVEN exists. The primary goal of treatment is the management of symptoms. Topical steroids and emollients may be prescribed for itching, while Calcipotriol may assist in preventing dryness in the affected area.