Presentation
[…] if the condition was present at the time of inpatient admission. [icd.codes]
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]
Here with we present a rare case in a newborn with Bullous congenital ichthyosiform erythroderma and to describe how the final diagnosis was obtained. [medresearch.in]
It presents at birth with generalized erythema, blisters, and erosions. [ommegaonline.org]
Present On Admission POA Help "Present On Admission" is defined as present at the time the order for inpatient admission occurs — conditions that develop during an outpatient encounter, including emergency department, observation, or outpatient surgery [icd10data.com]
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]
Case 2 The patient's 35-year-old mother gave a similar history of blistering and peeling of the skin since 1 month of age. As in her son, the blistering decreased with age and eventually ceased. [ijdvl.com]
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]
The newborn with blisters is important to rule out differential diagnosis mainly staphylococcal scalded skin syndrome, which is a toxin mediated skin condition that presents with blistering and wide spread erythema and exfoliation. [medresearch.in]
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]
- Ulcer
Acrodermatitis Cradle cap Cryotherapy Cutaneous skin tags Dry skin Dry skin -- self-care Epidermolysis bullosa Erythema multiforme Erythema nodosum Erythema toxicum Granuloma annulare Henoch-Schonlein purpura Ichthyosis vulgaris Intertrigo Ischemic ulcers [icdlist.com]
Skin Diseases Acrodermatitis Cryotherapy Cutaneous skin tags Dry skin Dry skin -- self-care Epidermolysis bullosa Erythema multiforme Erythema nodosum Erythema toxicum Granuloma annulare Henoch-Schonlein purpura Ichthyosis vulgaris Intertrigo Ischemic ulcers [coding-pro.com]
[…] congenital ichthyosiform erythroderma: a sporadic case produced by a new KRT10 gene mutation. ( 19689541 ) Betlloch I....Ballester I. 2009 45 Cutaneous malignant fibrous histiocytoma (undifferentiated pleomorphic sarcoma) arising in a chronic scalp ulcer [malacards.org]
EHK starts at birth with generalized erythroderma, blisters, and peeling with even mild trauma, leading to superficial ulcerations. [ommegaonline.org]
- Erythema Annulare
1 An interesting case of coincidental epidermolytic hyperkeratosis and erythema annulare centrifugum in the setting of latent tuberculosis in a 12-year-old female. ( 30623423 ) 2019 2 Identification and association of recurrent ALOXE3 mutation with non-bullous [malacards.org]
Treatment
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]
[…] 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. The scalp usually should be shampooed once a week, or if excessively oily, twice a week. Selenium sulfide shampoo suspension (Abbott) gives excellent results but tends to make the hair oily if used too frequently. [skindiseaselist.blogspot.com]
Topical treatment of ichthyotic disorders. Arch. Derm. 110, 586–590 (1974) PubMed Google Scholar 13. Peck, G.L., Yoder, F.W.: Treatment of lamellar ichthyosis and other keratinizing dermatoses with an oral synthetic retinoid. [link.springer.com]
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
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 [icdlist.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]
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]