We report phakomatosis pigmentovascularis (PPV) detected in a 40yearold male characterized by the presence of a port-wine stain in the background of aberrant Mongolian spots covering the back, nevus of Ota, ocular melanosis, epidermal nevus and a scaly patch with the diagnosis of discoid lupus erytematosus. These associations have not been reported yet.

Becker's nevus is a cutaneous hamartoma that may be present at birth, but more commonly is noticed during puberty. It classically manifests unilaterally on the shoulder and upper trunk as a tan to brown patch or thin plaque. "It typically has an irregular margin, breaks up into islands at the periphery, and has an average size of 125 square centimeters. Numerous skin, soft-tissue, and bony anom...

BACKGROUND The epidermal nevus syndromes include different diseases that have the common feature of mosaicism. One of these has been recently identified and named phacomatosis pigmentokeratotica, in analogy to phacomatosis pigmentovascularis. It is characterized by an organoid nevus with sebaceous differentiation, a speckled-lentiginous nevus, and other associated anomalies. It has been hypothe...

This study addresses the development of an animal model for human giant congenital nevomelanocytic nevi (GCNN). Skin grafts were made from 1) non-involved split-thickness skin from a 12-month-old GCNN patient, 2) nevus split-thickness skin from the same GCNN patient, 3) nevus full-thickness skin, and 4) cadaveric human split-thickness skin. For groups 1) and 2), human epidermal and dermal cells...

Epidermal nevus syndrome is a kind of neurocutaneous syndrome that is associated with epidermal nevus and a variety of congenital CNS disorders. Clinical presentations include seizures, paresis, mental retardation, and developmental delay. We report three cases with MR imaging and magnetoencephalography findings; one patient underwent ictal and interictal single photon emission CT. Both structu...

Epidermal Nevus Associated with Cerebral and Ocular MR Imaging Abnormalities This letter focuses on Schimmelpenning syndrome (SS), a neurocutaneous disorder related to epidermal nevus syndromes and characterized by craniofacial nevus, neurologic anomalies, and ocular pathology. We discuss clinical and MR imaging features of a 10-year-old boy, pointing out the etiopathologic substratum of this c...

Focal thickening of the calvarium, hypoplasia of the white matter, cortical calcifications, and a leptomeningeal drape that enhanced after contrast injection were demonstrated by MR in the parietooccipital region ipsilateral to a facial sebaceous nevus in a patient with epidermal nevus syndrome.

Melanocytes are pigment-producing cells derived from the neural crest. These specialized exocrine cells produce melanin, which is packaged and dispersed to neighboring keratinocytes in organelles called melanosomes. Within the melanocyte, tyrosine is converted to dopa, and then dopaquinone via the bifunctional enzyme tyrosinase. Dopaquinone is oxidized further to form the pigment melanin. Each ...

The "inflammatory linear verrucous epidermal nevus" is a rare disease, consisting of hyperplasia of the normal components of the epidermis. Its clinical features include erythematous and hyperkeratosic, warty, sometimes psoriasiform or lichenoid patches with a typical linear arrangement. At present, there are no effective medical therapies available. Currently, the best therapeutic results are ...

Inflammatory linear verrucous epidermal nevus (IL VEN) is a benign hyperplasia of the epidermis of unknown cause that most commonly occurs on the lower extremities.l ? The lesion usually appears in early childhood and is persistent and pruritic. Treatments for ILVEN have been associated with high rates ofrecurrence and/or scarring. This is the first report of successful treatment of ILVEN with ...