Presentation
Keratoderma, palmoplantar, non-epidermolytic (NEPPK) [MIM:600962]: A dermatological disorder characterized by well-demarcated hyperkeratosis is present over the palms and soles. A red band is frequently present at the periphery of the keratosis. [genecards.org]
It presents at birth with generalized erythema, blisters, and erosions. [ommegaonline.org]
Epidermolytic hyperkeratosis is a skin disorder that is present at birth. Affected babies may have very red skin (erythroderma) and severe blisters. [ghr.nlm.nih.gov]
Se realizo el diagnostico diferencial con otras enfermedades, como los condilomas acuminados • Clinical and light and electron microscopic observations of a 16-year-old male patient suffering from ichthyosis hystrix (Curth-Macklin) are presented. [scinapse.io]
[…] if the condition was present at the time of inpatient admission. [icd.codes]
Entire Body System
-
Amyloidosis
The pathological features are not specific for EHK, but can be seen in other benign and malignant dermatoses, such as pilar cysts, seborrheic keratosis, squamous cell carcinoma, verrucous keratosis, lichenoid amyloidosis, and granuloma annulare. [ommegaonline.org]
Skin
-
Blister
The name “epidermolytic hyperkeratosis” was introduced by Frost and Van Scott for the autosomal dominant blistering form of congenital ichthyosis [1]. It presents at birth with generalized erythema, blisters, and erosions. [ommegaonline.org]
It is characterised by blisters, skin fragility and ichthyosis. What does it look like? From birth the skin is noted to be fragile with blisters and peeling. [ichthyosis.org.uk]
Affected babies may have very red skin (erythroderma) and severe blisters. [ghr.nlm.nih.gov]
Epidermolytic Hyperkeratosis definition A form of congenital ichthyosis inherited as an autosomal-dominant trait and characterized by generalized blistering, erythroderma and severe hyperkeratosis.It is manifested at birth by widespread bullae followed [dermis.net]
Within a few weeks from birth, erythroderma and blister formation diminish and hyperkeratoses develop. [genecards.org]
Treatment
[…] repeated infections an unpleasant odour from the skin skin fragility and painful cracks in the skin creases thickening of the palms and soles is often a problem psychological upset with difficulties at school and work Treatment Moisturising creams and [ichthyosis.org.uk]
Treatment Medical Care Accurate diagnosis of epidermolytic ichthyosis (EI) is important in order to properly inform and counsel parents. [emedicine.medscape.com]
( 25751346 ) Jha A....Singh A. 2015 12 Linear Nevoid Epidermolytic Hyperkeratosis Localized to the Sole. ( 26790514 ) Khanna D....Bansal P. 2015 13 Epidermolytic Hyperkeratosis--case report. ( 26734873 ) Hayashida M.T....Mercante A.M. 2015 14 Dental Treatment [malacards.org]
A sudden episode of hemodynamic decompensation required insertion of an intra-aortic balloon pump and a continuous renal replacement treatment. He was upgraded to a status 1 condition. [journals.lww.com]
All issues pertaining to the care of patients with ichthyosis should be discussed with a dermatologist experienced in the treatment of their skin disorder. [firstskinfoundation.org]
Etiology
The etiology of seborrheic dermatitis is undetermined. That it is influenced by the relative amounts of androgens and estrogens in the body seems clear. [skindiseaselist.blogspot.com]
The fine scaling which was present in the face, neck and back was an important clue which supported as an ichthyotic etiology over staphylococcal scalded skin syndrome. [medresearch.in]
In this article, we have reviewed the histopathological appearance of these reaction patterns as well as their etiology and the conditions in which they are seen. [ijdpdd.com]
From the absence of the thyroid in his case of “ichthyosis congenita,” Winfield suggests further observation on this point as to possible etiologic rela tionship. Pa t h o l o gy . [doctortreatments.com]
Epidemiology
Relevant External Links for KRT1 Genetic Association Database (GAD) KRT1 Human Genome Epidemiology (HuGE) Navigator KRT1 Atlas of Genetics and Cytogenetics in Oncology and Haematology: KRT1 No data available for Genatlas for KRT1 Gene Frameshift mutation [genecards.org]
2B domain of KRT1. ( 23623204 ) Sung J.Y....Kim S.C. 2013 21 Systematized epidermal nevus with epidermolytic hyperkeratosis improving with topical calcipotriol/betametasone dipropionate combination ointment. ( 22938157 ) Koh M.J....Chong W.S. 2013 22 Epidemiology [malacards.org]
Prevention
Mutations in the KRT1 or KRT10 genes lead to changes in the keratin proteins, preventing them from forming strong, stable intermediate filament networks within cells. [ghr.nlm.nih.gov]
Your skin Holds body fluids in, preventing dehydration Keeps harmful microbes out, preventing infections Helps you feel things like heat, cold, and pain Keeps your body temperature even Makes vitamin D when the sun shines on it Anything that irritates [coding-pro.com]
Your skinHolds body fluids in, preventing dehydrationKeeps harmful microbes out, preventing infectionsHelps you feel things like heat, cold, and painKeeps your body temperature evenMakes vitamin D when the sun shines on itAnything ... [medicbind.com]