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Disorders of Keratinization and Other Genodermatoses
Published in Ayşe Serap Karadağ, Lawrence Charles Parish, Jordan V. Wang, Roxburgh's Common Skin Diseases, 2022
Roselyn Stanger, Nanette Silverberg
Overview: Epidermal nevus syndrome is an umbrella term for a variety of neurocutaneous syndromes. There are many congenital disorders that present with epidermal nevi in conjunction with various systemic manifestations, and multiple genes have been implicated, including FGFR3, HRAS, PIK3CA, PTEN, and AKT1, among others.
Developmental Diseases of the Nervous System
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
James H. Tonsgard, Nikolas Mata-Machado
Linear nevus sebaceous syndrome (LNSS) involves hypertrophic sebaceous gland nevus on the face or scalp, with mental retardation and seizures. It needs to be distinguished from the epidermal nevus syndrome.
A giant epidermal nevus of the face treated with a CO2 and dye laser combination: a case report and literature review
Published in Journal of Cosmetic and Laser Therapy, 2021
Mario Sannino, Azzurra Gaia Ambrosio, Giuseppe Lodi, Giovanni Cannarozzo, Luigi Bennardo, Steven Paul Nisticò
Epidermal nevi (EN) are benign, often focal hamartomas present or appearing shortly after birth and growing together with the child. Clinical lesions start as tan, velvety patches and then develop into hyperpigmented and papillomatous plaques. Histologically, these lesions show papillomatous hyperplasia of the epidermis and mature sebaceous glands in the dermis. EN is distributed along Blashko’s lines and are associated with defects in the ectoderm and mosaicism (1). When associated with other conditions (usually neurological issues), they constitute epidermal nevus syndrome. Giant epidermal nevus (GEN), when affecting the face and/or characterized by extensive body surface involvement, can often be psychologically devastating. EN therapy is still a challenge. Various therapies have been proposed to manage this condition, such as topical retinoids, topical and intralesional steroids, peelings, fluorouracil, podophyllin, cryosurgery, dermabrasion, and oral retinoids. These treatments have been associated with variable results, and a high risk of recurrence is usually present (2). Although resolutive, traditional surgery is usually associated with scarring and does not apply to large areas. Laser devices are becoming more and more exploited in the management of this condition (3). For the first time, we report using a combination technique between a surgical ablative laser and a vascular laser to treat a GEN.
Successful treatment of verrucous epidermal nevus with fractional micro-plasma radio-frequency technology and photodynamic therapy
Published in Journal of Cosmetic and Laser Therapy, 2018
Xinyao Zheng, Sijin He, Qian Li, Pingjiao Chen, Kai Han, Menglei Wang, Jia Guo, Menghua Zhu, Kang Zeng
First described by Baerensprung in 1963, verrucous epidermal nevus (VEN) is a skin disorder that commonly presents at birth (1). It is characterized by skin-colored to brown verrucous papules or papillomatous plaques, in a linear distribution following Blaschko’s lines, anywhere on the body; the papules and plaques can gradually increase in volume. It is an extremely rare and cosmetic disease, which is more common in females. Although extremely rare, VEN has been associated with malignant transformation such as basal or squamous cell carcinoma and keratoacanthoma. The “Epidermal Nevus Syndrome”, which is even more rare, is found in some individuals; it is characterized by complex developmental abnormalities of skin, eyes, nervous system, skeletal, urogenital, and cardiovascular systems. This disease leads to cosmetic problems, thereby reducing the quality of life. VEN has been treated by different treatment modalities, including topical 5-fluorouracil, topical calcineurin inhibitors, surgical excision, cryotherapy, photodynamic therapy (PDT), and laser therapy; however, its response to these treatments is varying and frustrating. Thus far, to the best of our knowledge, no reports on the use of fractional micro-plasma RF technology for the treatment of VEN has been presented. We combine the fractional micro-plasma RF technology and PDT to avoid scaring and rescurrence.