Basal cell nevus syndrome (BCNS, OMIM 109400) is a familial cancer characterized by the development of numerous basal cancers and various other developmental abnormalities, including epidermal cysts skin, calcified dural folds, keratocysts jaw, palmar plantar pits, ovarian fibromas, medulloblastomas, lymphomesenteric cysts, fetal rhabdomyomas. BCNS shows autosomal dominant inheritance caused mu...

Dear Editor, Becker nevus (BN) is characterized by a unilateral lesion consisting of hypermelanosis and hypertrichosis. It develops on the upper torso, shoulders, or arms, especially of male adolescents. Most cases are acquired, and the time of onset varies from infancy to adulthood. Although the etiology of this nevus is not yet known, an association with the expression of androgen receptors i...

Sturge-Weber syndrome can be associated with facial port-wine stains and intracranial calcification, and concurrent Klippel-Trenaunay-Weber syndrome has been reported. Klippel-Trenaunay-Weber syndrome is a rare congenital mesodermal phakomatosis characterized by cutaneous hemangiomas, venous varicosities and soft tissue or bone hypertrophy of the affected extremities. This report is presented a...

  Absract   Intracranial invasion of cellular blue nevus (CBN) from the skin is extremely rare   and such a condition with malignant transformation is even rarer. A case of   meningeal melanoma with malignant transformation which was derived from an Ota   nevus is presented in this report.   A21-year-old man with a neurocutaneous syndrome since childhood was referred   with headache and mild le...

BACKGROUND The diagnostic criteria of Proteus syndrome include various lesions of localized overgrowth such as digital gigantism, hemihyperplasia with unilateral macrocephaly, epidermal nevus, and mesodermal hamartomas such as lipoma, lymphangioma, hemangioma, or fibroma. Hyperplasia of the plantar dermal tissue may result in a characteristic cerebriform appearance. However, hypoplastic lesions...

Mutations in the tumor suppressor gene PATCHED (PTC) are found in human patients with the basal cell nevus syndrome, a disease causing developmental defects and tumors, including basal cell carcinomas. Gene regulatory relationships defined in the fruit fly Drosophila suggest that overproduction of Sonic hedgehog (SHH), the ligand for PTC, will mimic loss of ptc function. It is shown here that t...

Becker melanosis is an acquired melanosis and hypertrichosis usually in a unilateral distribution, first described in 1948 by S. William Becker. It is characterized by peripubertal development, male preponderance, hypertrichosis, occasional development of acneform lesions within the patch, and rare association with accessory scrotum in the genital region. Androgens may play a significant role i...

A 16-month-old female presented with an extensive epidermal nevus demonstrating epidermolytic hyperkeratosis on histologic evaluation. Individuals with this disorder are at increased risk of bearing children with epidermolytic ichthyosis. This occurs because the same mutations causing cutaneous somatic mosaicism may also affect the gonads. Genetic counseling is advised for individuals with exte...

neurocutaneous melanosis (ncm) is a rare, congenital non-hereditary syndrome, characterized by multiple pigmented nevi. we report the radiologic findings of a newborn who had extensive cutaneous melanotic nevus with satellite lesions in the brain. ultrasound showed multiple echogenic foci in the cerebral parenchyma. subsequent mri confirmed these lesions as characteristic deposits of melanin. t...