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 ...

Porokeratosis is a genodermatosis characterized by abnormal epidermal keratinization with the histological feature of cornoid lamella. There are many clinical variants, but two or more of these variants rarely occur in a single patient. This variation in clinical presentation may be due to the different phenotypic expression of a common genetic abnormality or may be the consequence of abnormali...

A Japanese baby was born with a polypoidal projection with hair, on the lateral corner of upper eyelid. The tumour had extent to bulbar sub-conjunctival area. He presented no other malformation nor pathological symptoms. There was no sign of association with Goldenhar, hemifacial macrosomia, epidermal nevus or linear nevus sebaceous syndromes. The tumour was resected at his age of 4 months. The...

Corresponding author: Hsiu-Cheng Hsu, Department of Dermatology, Chang Gung Memorial Hospital, No. 199, Tunghwa N Rd., Taipei, 105, Taiwan TEL: 886-2-27135211 ext. 3397 FAX: 886-2-27191623 E-mail: [email protected] Funding source: none Confl ict of interest: none declared CASE REPORT A 53-year-old man presented with a 20year history of asymptomatic, progressive pigmented thickening of hi...