Title : A report of periocular Epidermodysplasia Verruciformis lesions in a Young Adult with a Review of Literature
Abstract:
Epidermodysplasia Verruciformis (EV) is a rare autosomal recessive dermatological genetic disorder of the immune system, primarily affecting the extremities of the limbs. Presentations with involvement of the face and other regions of the body is rare, especially the periocular region. We report a 19-yr-old young adult with history of recurrent warty lesions of the extremities since eight years of age with involvement of the periocular region for about two years, managed with surgery and followed up for 2 years with no clinical recurrence. Ophthalmic examination revealed bilateral periocular multiple verrucous papules and plaques. He also had extensive lesions of the dorsum of both hands and feet, and of the other areas of the face. Ophthalmologically, he underwent surgical excision of the periocular lesions, the largest of which was 24 x 16 mm in size. Parents were non consanguineously married, however had a family history of his older sister with similar findings. Histopathological examination showed hyperkeratosis, papillomatosis tiers of parakeratosis with hyper granulosis, irregular acanthosis with elongated, bulbous, and anastomosing rete pegs. Superficial dermis showed proliferative blood vessels with perivascular infiltrates of lymphocytes and histiocytes. Interprofessional team consultations from Dermatology and Medical Genetics were obtained. The patient was initiated on systemic Retinoids for his symptomatic relief and to improve his cosmetic disfigurement. There was no recurrence noted till 2 years of follow up following the surgical excision. Epidermodysplasia Verruciformis, though has no definitive and permanent curative therapies available, a combination of treatment modalities along with multidisciplinary team consultation can provide an improved quality of life for the affected individuals. Patients with EV require lifelong psychological support, counselling and periodic surveillance to monitor recurrence or malignant transformation of the lesions.
