Epidermal nevus syndrome (ENS) represents a diverse group of rare neurocutaneous diseases associated with the presence of characteristic epidermal nevi (EN) in the skin and extracutaneous manifestations in the eyes, skeletal, urogenital and central nervous systems. We present a case series of 7 children with ENS, with specific attention to the neuroradiological characteristics of this entity.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7719931 | PMC |
| http://dx.doi.org/10.21037/qims-20-634 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
A case of subcutaneous metastatic malignant melanoma of the left medial ankle: a case report and review of literature.
J Med Case Rep
December 2024
College of Medicine and Life Sciences, Division of Plastic and Reconstructive Surgery, University of Toledo, 3000 Arlington Ave, Toledo, OH, 43614, USA.
Background: Although rare, melanoma confined to the dermis or subcutaneous tissue without evidence of a primary cutaneous site should provoke consideration of melanoma of unknown primary. This diagnosis carries a favorable prognosis when compared with cutaneous metastatic melanoma. Several hypotheses have been proposed for how melanoma of unknown primary develops, two of which were considered in our patient case: (1) spontaneous regression of the primary tumor following metastasis or (2) the traumatic implantation of ectopic melanocytic cells in other tissues, such as the subcutaneous tissue.
View Article and Find Full Text PDFAssociation of Schimmelpenning Syndrome with Astrocytoma (WHO Grade 3): Case Report.
Medicina (Kaunas)
October 2024
Department of Neurology and Neurosurgery, Riga Stradins University, 1007 Riga, Latvia.
Schimmelpenning syndrome, or epidermal nevus syndrome, is a rare, neurocutaneous disorder characterized by skin abnormalities, such as epidermal nevi, and involvement of the central nervous system, including intracranial tumors. There are only a few reported cases of intracranial tumors associated with Schimmelpenning syndrome. In most cases, a single nucleotide mutation in the RAS family proto-oncogenes, like or genes, can result in the genetic mosaicism that is responsible for the clinical manifestations of this syndrome.
View Article and Find Full Text PDFA Case of Systematized Epidermal Nevus (Nevus Unius Lateris) in a 20-year-old Filipino Female Treated with Ablative CO Laser and Topical Tretinoin.
Acta Med Philipp
September 2024
Department of Dermatology, Philippine General Hospital, University of the Philippines Manila.
This is a case of a 20-year-old female with no known comorbidities presenting with verrucous plaques arranged in a unilateral blaschkoid distribution at birth. Biopsy was consistent with epidermal nevus hence patient was diagnosed as systematized epidermal nevus, Nevus Unius Lateris type. Gold standard treatment is full thickness surgical excision however, due to the extensive involvement, treatment of this condition remains a challenge.
View Article and Find Full Text PDFEccrine Porocarcinoma in Linear Epidermal Nevus.
Clin Cosmet Investig Dermatol
October 2024
Department of Dermatology, Beijing Hospital, National Center of Gerontology, Institute of Geriatric Medicine, Chinese Academy of Medical Sciences, Beijing, People's Republic of China.
Article Synopsis
- Linear epidermal nevus is a type of skin disorder that is typically present at birth or develops during childhood.
- It involves abnormal skin growths that resemble moles or patches.
- The case presented discusses a rare occurrence where this skin condition was followed by a serious skin cancer called eccrine porocarcinoma (EPC).
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!