Inflammatory Linear Verrucous Epidermal Nevus (ILVEN): A case report of unusual presentation

Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare variant of epidermal nevus (EN). EN is benign hamartomatous skin disease characterized by hyperplasia of epidermal structures. Here in we report a 5-year-old boy, presented with history of persistent and slowly progressing very itchy skin lesions that appeared few months after birth. Skin examination revealed multiple linear scaly keratotic skin colored plaques on his right upper extremity and right side of his trunk that are following Blaschko lines. Skin biopsy revealed alternating orthokeratosis and parakeratosis. The parakeratosis overlies a zone of hypogranulosis, whereas the orthokeratosis overlies a zone of hypergranulosis. The patient was diagnosed as ILVEN. He was prescribed potent topical steroid ointment and keratolytics. Our case is unusual in that it is a boy with trunk involvement.