Presentation
The present illustration emphasizes the importance of a punctilious work up of the case. [ncbi.nlm.nih.gov]
A 12-year-old girl presented with extensive epidermal nevi, new onset seizures, mental retardation, and oral and ocular abnormalities. [ucdavis.pure.elsevier.com]
Entire Body System
- Short Stature
In children, growth rates may be slow, ultimately resulting in short stature. Affected individuals may be prone to fractures. [rarediseases.org]
- Swelling
Surgical exploration revealed numerous polypous nevi, external ear cholesteatoma, and tumorous swelling of the facial nerve. The middle ear ossicles were completely lost. [ncbi.nlm.nih.gov]
Cryosurgery has similar limitations, with the risks including slow healing, infection, swelling, and not uncommonly, abnormal coloration of the skin. Physicians have been performing laser treatment on epidermal nevi for decades. [dermatopedia.com]
In 2001, a large study confirmed the importance of Breslow thickness as one of the three most important prognostic factors in melanoma, along with tumor (T) stage and the existence of ulceration (broken skin, bleeding, swelling). [slideplayer.com]
[…] containing an overgrowth of sebaceous glands, frequently growing larger during puberty or early adult life, and rarely giving rise to a variety of new growths, including basal cell carcinoma. nevus vascularis, nevus vasculosus, vascular nevus a reddish swelling [medical-dictionary.thefreedictionary.com]
Excessive amounts of fluid accumulate around the brain and cause abnormally high pressure within the skull, swelling of the head (congenital hydrocephalus), and neurological impairment. Motor delays and learning problems may also occur. [rarediseases.org]
- Myxedema
Granuloma Leukocytoclastic vasculitits Lichen planus Lichen Simplex chronicus Lower extremity vascular insufficiency Mast cell diesase Mast cell disease: Degranulation Blockers Morphea Myxedema Necrobiosis Lipoidica Necrobiotic Xanthogranuloma Neutrophilic [euro-libris.ro]
Skin
- Cafe-Au-Lait Spots
Hemangiomas, lipomas, hypopigmented lesions, and rarely cafe΄ au lait spot have also been described. Among central nervous system (CNS) abnormalities, unilateral HME is the most common finding and is typically ipsilateral to the skin lesions. [annalsofian.org]
There were no other cutaneous manifestations, such as cafe-au-lait spots or hypo- or hyper-pigmentation lesions. The infant appeared healthy otherwise with no apparent neurologic deficit on physical examination. [jcnonweb.com]
- Skin Disease
None of her siblings or parents had history of any skin disease. Drug history of mother during pregnancy was not significant. General physical examination and systemic examination were within normal limits. [edoj.org.eg]
Neurologic
- Convulsions
Recently we had the opportunity of studying 2 patients with unusual linear nevi of the face associated with convulsions and mental retardation. [doi.org]
It was the report by Feuerstein and Mims of 2 children with a linear sebaceous nevus in the midline of the face (confirmed by skin biopsy) associated with epilepsy and mental retardation under the explicit title “Linear nevus sebaceous with convulsions [medlink.com]
- Giddiness
She had focal dyscognitive seizures with secondary generalization with semiology consisting of transient giddiness followed by vacant blank stare, loss of contact with surrounding, which was associated with deviation of eyes to the left side and tonic [pediatricneurosciences.com]
Workup
This case highlights the necessity of appropriate workup in patients presenting with epidermal nevi at any age. [ncbi.nlm.nih.gov]
Christian Hafner, University of Regensburg, Germany towards the diagnosis and workup of this patient. 1. Happle R. Epidermal nevus syndromes. Semin Dermatol 1995;14:111-21. 2. Vujevich JJ, Mancini AJ. [e-ijd.org]
Workup Imaging Studies MRIs can be used to evaluate intracranial involvement. MRIs may show cerebral atrophy, dilated ventricles, hemimegalencephaly, pachygyria, or enlarged white matter. [dermaamin.com]
Treatment
Treatment of the epidermal nevus may be nonoperative, differing from the philosophy of aggressive treatment of the similarly appearing premalignant congenital hairy nevus. [ncbi.nlm.nih.gov]
The treatment of epidermal nevi is challenging. Multiple medical and surgical treatments have been attempted, but no ideal or universally acceptable treatment has emerged. [dermatopedia.com]
Prognosis
Prognosis The prognosis depends on the severity of the clinical manifestations and the extent of the multisystem involvement. The documents contained in this web site are presented for information purposes only. [orpha.net]
There is one instance in which Clark’s levels continue to be used to predict prognosis: in patients with thin (less than 1.0 mm) melanoma, a Clark’s level IV or V lesion portends a worse prognosis. [slideplayer.com]
What is the Prognosis of Epidermal Nevus? (Outcomes/Resolutions) The prognosis of Epidermal Nevus is good, especially with a surgical approach. [dovemed.com]
Prognosis The prognosis is very good. [patient.info]
The specific symptoms present, severity and prognosis can vary greatly depending on the specific type of ENS and the presence and extent of associated extra-cutaneous symptoms. The onset and progression of these disorders varies greatly as well. [rarediseases.org]
Etiology
They both exhibit overlapping and distinctive features but same etiology: post-zygotic mosaic mutations in RAS genes. [ncbi.nlm.nih.gov]
Etiology LNSS is a sporadic disease. It is hypothesized that the epidermal nevus syndromes are the result of genetic mosaicism involving a dominant gene. [orpha.net]
(Etiology) The majority of Epidermal Nevi (plural of Nevus) are sporadic, i.e. they occur after birth, without a genetic basis. [dovemed.com]
Epidemiology
The discussion covers the phenotype spectrum, epidemiology, mode of inheritance, pathogenesis, and clinical profile of each condition, all of which is accompanied by a wealth of illustrations. [books.google.com]
Summary Epidemiology The incidence of sebaceous nevi at birth is estimated at 1 in 1000 but the prevalence of these lesions in association with other anomalies, as in the case of LNSS, varies between case reports to around 1:10,000. [orpha.net]
This report defines the prevalence of genital colonization in a large population of women, characterizes the women with S. aureus, and describes epidemiologic features of genital carriage. Página 50 - Lautenschlager S, Eichmann A. [books.google.es]
Methods Statistics Trigonometry Medical & Nursing Anatomy Anesthesiology Audiology Bacteriology Biochemistry Bioethics Biomedical Science Cardiology Cardiovascular Childbirth Chiropractic Dentistry Dermatology Diagnostic Imaging Drugs Endocrinology Epidemiology [brainscape.com]
Epidemiology Nevus sebaceous occurs in approximately 0.3% of all newborn infants [1]. Both sexes are equally affected [5]. There is no racial and⁄ or ethnic predominance [2]. [austinpublishinggroup.com]
Pathophysiology
Export citation Add to favorites Track citation Summary This chapter contains sections titled: Lentigo Simplex Lentigo Senilis et Actinicus Centrofacial Lentiginosis LEOPARD Syndrome Carney Complex Other Lentiginoses The Pigmentary System: Physiology and Pathophysiology [onlinelibrary.wiley.com]
& Vitamin K Antagonists: Anticoagulation Affects on the Clotting Cascade Wilson’s Disease Section 9 - Genodermatoses and Syndromes Addison’s Disease Amyloid Basal Cell Nevus Syndrome Carney Complex Cushing's Syndrome and Disease Cushing's Syndrome: Pathophysiology [euro-libris.ro]
Pathophysiology Epidermal nevi arise from pluripotential germinative cells of the basal layer of the embryonic epidermis. [dermaamin.com]
Prevention
Surgical removal has proven to be the most successful treatment method; though, it is associated with certain risks How can Epidermal Nevus be Prevented? Currently, there are no known means of preventing Epidermal Nevi occurrence. [dovemed.com]
Atlanta: Centers for Disease Control and Prevention, September 1995. Página 51 - Stone KM, Reiff-Eldridge R, White AD, Cordero JF, Brown Z, Alexander ER, Andrews EB. [books.google.es]
Transplantation Substance Use and Addiction Surgery Surgical Innovation Surgical Pearls Teachable Moment Technology and Finance The Rational Clinical Examination Tobacco and e-Cigarettes Toxicology Trauma and Injury Treatment Adherence United States Preventive [jamanetwork.com]
Doctors recommend proper protection from the sun (like sun block) to prevent skin cancers and to reduce development of acquired nevi. Living & Managing Atypical lesions or lesions associated with malignancies require close follow-up from a doctor. [texaschildrens.org]