Bullous ichthyosiform erythroderma typically presents with fragile skin (epidermolysis), which gives way to progressive hyperkeratosis. A mild, generalized erythroderma is present at birth. [ijdvl.com]

SEI presents at birth or during the neonatal period with mild superficial blistering that is more frequent on flexures, shins, abdomen and extremities. [orpha.net]

Initially, EBSS was considered based on AD inheritance, the generalised distribution, and lack of inflammation and ichthyosis at presentation. [adc.bmj.com]

At birth, patients present with generalized erythroderma, characterized by erythema and scaling, with or without edema over 90% of the skin [7]. Erosions, blisters, and peeling are typically present [7]. [austinpublishinggroup.com]

Definition / general Keratinization disorder which presents at birth with generalized erythema, widespread bullae and desquamation resulting in denuded skin Form of ichthyosis, an abnormality of epidermal maturation resulting in skin fragility "Epidermolytic [pathologyoutlines.com]

Management and treatment Treatment is symptomatic. Emollient and mild topical keratolytics may be used to reduce hyperkeratosis. [orpha.net]

Readers are guided through the maze that too often lies between the presenting skin complaint and its final diagnosis and treatment. [books.google.com]

The rabbit came as a second opinion from another practice where she was treated for mange mites for a month with Doramectin and a month treatment for dermatophytes without result of both treatments. [bavd.bg]

Prognosis SEI is usually a mild disease. Life expectancy is normal and quality of life is not severely impaired. The documents contained in this web site are presented for information purposes only. [orpha.net]

[…] individuals often have darker scales Associations - up to 50% of patients with ichthyosis vulgaris have features of atopic eczema, and many have keratosis pilaris Skin creases on the palms and soles are more prominent and there can be painful fissuring Prognosis [pcds.org.uk]

She advised clinicians to obtain a sample soon after birth to confirm the clinical diagnosis, assist with assessing prognosis, and enable genetic counseling. “It’s important to help identify those at risk for systemic complications,” she said. [mdedge.com]

Prognosis After successful treatment of his arm, Mr. M was anxious to continue treatment on the other affected areas of his body. [o-wm.com]

Describe the presentation and etiology of epidermolytic hyperkeratosis. Identify and describe the two major autosomal recessive primary ichthyoses and their etiologies. the primary technique for dry skin care. [pedsinreview.aappublications.org]

Etiology SEI is caused by mutations in the KRT2 gene encoding keratin 2. These mutations impair keratin filament formation and weaken the structural stability of the keratinocyte cytoskeleton. [orpha.net]

AB - Background: Inherited ichthyoses belong to a large, clinically and etiologically heterogeneous group of mendelian disorders of cornification, typically involving the entire integument. [ohsu.pure.elsevier.com]

Ichthyosis: etiology, diagnosis, and management. Am J Clin Dermatol. 2003;4:81-95. Schmuth M, Yosipovich G, Williams ML, et al. Pathogenesis of the Permeability Barrier Abnormality in Epidermolytic Hyperkeratosis. J Inves Derm. 2001;17(4):837-847. [rarediseases.org]

Keratosis palmoplantaris striata Synonym(s): - Ichthyosis bullosa of Siemens - SEI Classification (Orphanet): - Rare genetic disease - Rare skin disease Classification (ICD10): - Congenital malformations, deformations and chromosomal abnormalities - Epidemiological [csbg.cnb.csic.es]

Summary Epidemiology Less than 30 families have been reported in the literature. Clinical description Clinical features of SEI are similar but milder that those of epidermolytic ichthyosis (EI; see this term). [orpha.net]

[Crossref] Kurosawa M, Takagi A, Tamakoshi A, Kawamura T, Inaba Y, et al. (2013) Epidemiology and clinical characteristics of bullous congenital ichthyosiform erythroderma (keratinolytic ichthyosis) in Japan: Results from a nationwide survey. [oatext.com]

Another limitation of the study was the inclusion of patients seen in an expert hospital reference center for ichthyosis where the most severe forms are seen (28% of severe or very severe forms vs 23.5% in a recent French epidemiological study [ 28 ]) [ojrd.biomedcentral.com]

Various tables provide an overview on clinical and/or pathophysiologically related groups of diseases. [rooksdermatology.com]

[…] epidermolytic leukoplakia Terminology Other names: Epidermolytic ichthyosis Bullous congenital ichthyosiform erythroderma Bullous ichthysosis Bullous erythroderma ichthyosiformis congenita of Brocq Disorder of cornification type 3 Vorner's syndrome Pathophysiology [pathologyoutlines.com]

Further pathophysiological study should be performed to explore this hypothesis. One important additional point that needs to be discussed is the impact of sun avoidance on vitD level in ichthyosis patients. [ojrd.biomedcentral.com]

[…] minus' sign Virchow -triad Use Parentheses water AND (cup OR glass) Add an asterisk (*) at end of a word to include word stems Neuro* will search for Neurology, Neuroscientist, Neurological, and so on Use quotes to search for an exact phrase "primary prevention [read.qxmd.com]

[…] able to prevent only the abnormal keratin protein from being formed. [firstskinfoundation.org]

[…] and preventing normal cellular function). [o-wm.com]

Home Health Centers Wellness, Prevention & General Health Arthritis Asthma & Allergies Child & Adolescent Health Diabetes Digestive Disorders / GERD Emotional Well-Being Heart Disease High Cholesterol Hypertension Pain Disorders Men's Health Senior Health [drdoleary.com]

The disease may also interfere with the person's skin and its ability to protect against infection, regulate body temperature, and prevent dehydration. [disabled-world.com]