Giant congenital melanocytic nevus is a skin condition characterized by an abnormally dark, noncancerous skin patch (nevus) that is composed of pigment-producing cells called melanocytes. It is present from birth (congenital) or is noticeable soon after birth. The nevus may be small in infants, but it will usually grow at the same rate the body grows and will eventually be at least 40 cm (15.75 inches) across. The nevus can appear anywhere on the body, but it is more often found on the trunk or limbs. The color ranges from tan to black and can become darker or lighter over time. The surface of a nevus can be flat, rough, raised, thickened, or bumpy; the surface can vary in different regions of the nevus, and it can change over time. The skin of the nevus is often dry and prone to irritation and itching (dermatitis). Excessive hair growth (hypertrichosis) can occur within the nevus. There is often less fat tissue under the skin of the nevus; the skin may appear thinner there than over other areas of the body.
NRAS gene mutations cause most cases of giant congenital melanocytic nevus. Rarely, mutations in the BRAF gene are responsible for this condition.
Giant congenital melanocytic nevus occurs in approximately 1 in 20,000 newborns worldwide.
This condition is generally not inherited but arises from a mutation in the body's cells that occurs after conception. This alteration is called a somatic mutation. A somatic mutation in one copy of the NRAS or BRAF gene is sufficient to cause this disorder.
Veronica Kinsler practices in London, United Kingdom. Kinsler is rated as an Elite expert by MediFind in the treatment of Giant Congenital Melanocytic Nevus. She is also highly rated in 24 other conditions, according to our data. Her top areas of expertise are Giant Congenital Melanocytic Nevus, Mosaicism, Phacomatosis Pigmentovascularis, and Ectodermal Dysplasias.
Satyamaanasa Polubothu practices in London, United Kingdom. Polubothu is rated as an Elite expert by MediFind in the treatment of Giant Congenital Melanocytic Nevus. They are also highly rated in 10 other conditions, according to our data. Their top areas of expertise are Giant Congenital Melanocytic Nevus, Mosaicism, Phacomatosis Pigmentovascularis, and Linear Nevus Sebaceous Syndrome.
Sven Krengel practices in Lübeck, Germany. Krengel is rated as an Elite expert by MediFind in the treatment of Giant Congenital Melanocytic Nevus. He is also highly rated in 7 other conditions, according to our data. His top areas of expertise are Giant Congenital Melanocytic Nevus, Neurocutaneous Melanosis, Melasma, and Ectodermal Dysplasias.
Background: Oral hedgehog inhibitors vismodegib and sonidegib have been used for the treatment of locally advanced (laBCC), metastatic basal cell carcinoma (mBCC) and in basal cell nevus syndrome (BCNS) patients. In the Netherlands, targeted therapy with vismodegib and sonidegib has been available since 2013 and 2021, respectively. No direct comparative studies have been performed between the two oral hedgeho...
Summary: The primary objectives are to: Evaluate the safety and tolerability of intralesional ASN-002 when administered in combination with oral vismodegib in patients with Basal Cell Carcinomas (BCC)s; Evaluate the efficacy of intralesional ASN-002 in target tumours when administered in combination with oral vismodegib in patients with BCCs. The secondary objective is to: 1) Evaluate the efficacy of intra...
Published Date: December 01, 2014Published By: National Institutes of Health