Presentation
The present illustration emphasizes the importance of a punctilious work up of the case. [ncbi.nlm.nih.gov]
Entire Body System
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Pseudotumor
The first reported case of facial paralysis due to an inflammatory pseudotumor (IPT) of the facial nerve as a complication of epidermal nevus syndrome (ENS) is herein presented. [ncbi.nlm.nih.gov]
Musculoskeletal
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Advanced Bone Age
Idiopathic central precocious puberty characterized by premature breast and public hair development and advanced bone age developed at the age of 20 months. [ncbi.nlm.nih.gov]
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Bone Pain
Clinical symptoms were marked bone abnormalities, muscle weakness, and bone pain. [ncbi.nlm.nih.gov]
Eyes
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Retinal Vascular Anomaly
Her hearing was impaired because of serous otitis media and her visual acuity decreased because of hard yellow exudate in the macula as a result of decompensation and plasma leakage from the peripheral retinal vascular anomalies. [ncbi.nlm.nih.gov]
Psychiatrical
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Psychomotor Retardation
Epidermal nevus syndrome is defined as a combination of nevoid skin alterations, epileptic seizures, and psychomotoric retardation. Involvement of other organs, especially the eyes, may also occur, but is not obligatory. [ncbi.nlm.nih.gov]
Onset
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Neonate-Onset
We found 57 previously reported cases with the hemimegalencephalic variant of epidermal nevus syndrome, in which the most frequent associated features are severe epilepsy, in about half of cases with neonatal onset, mental retardation/developmental delay [ncbi.nlm.nih.gov]
Neurologic
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Febrile Seizures
A 13-year-old boy with the neurologic variant of ENS with hemimegalencephaly, facial asymmetry, febrile seizures and mental retardation is reported. [ncbi.nlm.nih.gov]
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]
EEG
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Hypsarrhythmia
Electroencephalograms showed a suppression-burst pattern in the neonatal period, subsequently changing to hypsarrhythmia. [ncbi.nlm.nih.gov]
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Focal Spikes
Interictal EEG occasionally depicted spontaneous focal spikes and waves in the left frontotemporal area, and ictal EEG depicted vertex spikes and then diffuse slow spike-and-wave complex bursts. [ncbi.nlm.nih.gov]
Serum
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Hyponatremia
The clinical picture of hypotonic hyponatremia, high urine osmolality, elevated urine sodium, and euvolemia was compatible with SIADH. The seizures did not correlate with the hyponatremia, and no other cause for the seizures could be identified. [ncbi.nlm.nih.gov]
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]
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]
Prognosis The prognosis is very good. [patient.info]
Recognition of Heide's syndrome can help physicians plan a multidisciplinary approach to investigation, management, and prognosis. [medlink.com]
What is the Prognosis of Epidermal Nevus? (Outcomes/Resolutions) The prognosis of Epidermal Nevus is good, especially with a surgical approach. [dovemed.com]
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
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]
A detailed review of the genetic basis and pathogenesis of ENS is available. [ 2 ] Visual appearance A pigmented, linear example of an epidermal naevus Epidemiology Epidermal naevi (with or without other organ involvement) have an incidence of around [patient.info]
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
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]
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]
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]