Neurocutaneous melanosis (NCM) is a rare condition characterized by central nervous system melanocytic tumors associated with congenital melanocytic nevi. Phacomatosis pigmentovascularis (PPV) is an association of vascular nevus with pigmentary nevus. Aberrant maturation of neural crest-derived cells is considered to be related to pathogenesis in both conditions. However, association of NCM and...

BACKGROUND Congenital melanocytic nevus is a benign proliferation of melanocytes that is present at birth or develops in the first months of life. Although such lesions are common, few studies have analyzed their prevalence in Spain. OBJECTIVES The aim of this study was to define the prevalence and most frequent anatomical areas affected by congenital melanocytic nevi in our health service ar...

Phakomatosis pigmentovascularis (PPV) is a rare congenital malformation syndrome that is characterized by a combination of capillary abnormalities and dermal melanocytosis.We describe 3 cases of PPV combined with bilateral Sturge-Weber syndrome (SWS), Ota nevus, and congenital glaucoma.Case 1 was a 2-year-old boy. Facial port-wine stains distributed along the 3 branches of his trigeminal nerves...

Basal cell nevus syndrome Congenital hydrocephaly Abstract A case of basal cell nevus syndrome or Gorlin’s syndrome is reported in a new-born. The skin condition is associated with congenital hydrocephaly and skeletal malformations. To our knowledge, this is the first case of basal cell nevus syndrome with skin tumors present at birth and localized on the fingers K. Meyvisch, Department of Derm...

Nevus (or mole) is a form of skin cancer that begins in melanocytes, the cells responsible for skin pigmentation. If it is present at birth, it is called congenital nevus. Before adolescence, nevi are regularly distributed throughout the body, with very few Large nevi (> 5 mm) prevailing on the trunk (due to brutal sun exposure) while those on the face and limbs, chronically exposed to moderate...

Congenital and infantile malignant melanomas are rare and typically carry poor prognosis. The purpose of this article was to review the data on congenital and infantile malignant melanomas of the scalp in order to understand its presentation, diagnosis, management, and outcomes of congenital melanoma of scalp. We searched PubMed, CINAHL and Cochrane databases. Ten cases of congenital and 3 case...

Figure 1. Two-year-old boy with a giant congenital melanocytic nevus of the left buttock. Left, Center, Preoperative views. Right, Immediate view after excision and placement of the Integra bilayer matrix dressing. Figure 2. Left, Wound after removal of the silicone layer of the Integra bilayer matrix dressing. Right, Coverage of the wound with a split skin autograft.

Pedunculated lipofibroma is a rare benign hamartomatous condition that is characterized by ectopic adipose tissue in the dermis and a solitary form of nevus lipomatosus cutaneous superficialis. We describe here a neonate with congenital pedunculated lipofibroma. The lesion was removed by simple excision.