epidermal nevus syndrome is a rare congenital disorder, characterized by epidermal nevi and multiple organ involvement. multicystic kidney disease has been very rarely reported in this syndrome. here is the report of a boy presented with multiple epidermal nevi, cardiac anomaly, seizure attack, hemi hypertrophy, and multicystic dysplastic kidney complicated with wilms' tumor. according to this ...

Epidermal nevus syndrome (ENS) is a rare condition characterized by congenital epidermal nevi (EN) associated abnormalities of other organs including central nervous system (CNS), skeletal system, eyes, and oral cavity (1). ENS is divided into a group of distinct disorders, based on the associated epidermal nevus and genetic inheritance pattern. Organoid nevus could be seen in some ENS, includi...

Hamartomas represent localized overgrowth of cells in the parts which are normally associated with polyps, ie. mesenhimal, stromal, endodermal and ectodermal elements. Hamartomatous polyposis syndromes carry a significant risk of developing dysplasia, adenomas, gastrointestinal carcinomas, and pancreatic carcinomas. These syndromes may be classified on the basis of whether they represent heredi...

Cutaneous symptoms in some patients with clinical picture of Schimmelpenning-Feuerstein-Mims syndrome can include a speckled lentiginous nevus, also known as nevus spilus. Recent investigations show that somatic heterozygous HRAS mutations are present in the sebaceous and speckled lentiginous nevus tissues of patients with combination of two nevi.

The lesion traditionally known as odontogenic keratocyst has been renamed by WHO in 2005, as “keratocystic odontogenic tumor” as it is more appropriate and reflects its potential for local, destructive behavior. It is a benign intraosseous neoplasm of jaw, which is unusual due to its characteristic histopathological and clinical features, including potentially aggressive behavior, high recurren...

1. Sugarman JL. Epidermal nevus syndromes. Semin Cutan Med Surg. 2007;26:221-30. 2. Solomon LM, Fretzin DF, Dewald RL. The epidermal nevus syndrome. Arch Dermatol. 1968;97:273-85. 3. Solomon LM, Esterly NB. Epidermal and other congenital organoid nevi. Curr Provi Pediatr. 1975;6:1-56. 4. Singal A, Dhaliwal U, Bhattacharyya SN , Rohatgi J, Singh N. Complex ocular choristomas in linear nevus seba...

skin cancer is one of the most important type of cancers in the world. in this way, molecular investigation in order to detect some novel mechanisms and polymorphisms involved in cancer development can be impressive and vital. in this way, the aim of this study was the histopathological investigation of skin cancer and its relationship with polymorphism of cdkn2a gene. this case-control study w...