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 ...

Nevus sebaceus of Jadassohn is a congenital cutaneous hamartoma comprised of multiple skin structures. It has the potential to develop into variety of neoplasms of various epidermal adnexal origins. While multiple tumors may occasionally arise, it is unusual for more than four tumors to arise simultaneously within a single sebaceus nevus. Here in, we report a case of a 70-year-old woman with si...

Since 1994, four cases of epidermal nevus with epidermolytic hyperkeratosis (EH) caused by keratin 10 gene mutations have been reported, although no keratin 1 (K1) gene mutation has yet been reported. We detected a K1 gene (KRT1) mutation in epidermal nevus with EH in a 10-year-old Japanese male. The patient showed well-demarcated verrucous, hyperkeratotic plaques mainly on the trunk, covering ...

A six year old female child with generalized hyperpigmented hyperkeratotic verrucous plaques with nail and dental abnormalities suggestive of ichthyosis hystrix type of epidermal nevus is being reported in view of the rarity of this condition.

261 We appreciate Dr. Yu Jin Kim for her insightful commentary on our recent article [1]. The additional masses that are mentioned in Dr. Yujin Kim’s commentary were attached to the two masses shown on the left side of the gross specimen figure, each with its own epidermal layer [1,2]. The epidermal cyst in the center of the coronal computed tomography image is that which had the foreign body r...

We report a 4-year-old boy with two areas of woolly hair in the right parietotemporal region and a linear epidermal nevus in the areas of woolly hair as well as in the ipsilateral hemiface and chin. Evaluation by scanning electron microscopy showed woolly hair with oval transverse section and longitudinal groove. A complete examination ruled out associated anomalies.