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.

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...

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...

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 ...

Nevus cerebriforme gigante Oscar Duménigo Arias,* Yudisley Guerrero Osorio, Yamila Valiente Hernández c a Servicio de Cirugı́a General, Hospital Universitario Clı́nico Quirú rgico «Amalia Simoni», Camagüey, Cuba b Servicio de Medicina General Integral, Policlı́nica Comunitaria Docente «René Ávila Reyes», Holguı́n, Cuba Servicio de Dermatologı́a, Hospital Universitario Clı́nico Quirú rgico «Juan Bruno...