Topical polyhydroxy acid treatment for autosomal recessive congenital ichthyosis in the golden retriever: a prospective pilot study.

Topical polyhydroxy acid treatment for autosomal recessive congenital ichthyosis in the golden retriever: a prospective pilot study. Vet Dermatol. 2018 May 22;: Authors: Puigdemont A, Furiani N, De Lucia M, Carrasco I, Ordeix L, Fondevila D, Ramió-Lluch L, Brazis P Abstract BACKGROUND: Autosomal recessive congenital ichthyosis (ARCI) in golden retrievers is due to a PNPLA1 gene mutation, which plays a role in epidermal lipid organization and metabolism. Topical therapies are used to reduce scaling; however, there are few published efficacy studies. OBJECTIVES: To examine the efficacy of topical treatment based on gluconolactone, a polyhydroxy acid with known beneficial effects on stratum corneum structure. ANIMALS: Sixteen golden retriever dogs with clinical signs of ARCI and PCR-confirmed PNPLA1 gene mutation. METHODS: This was a prospective, multicentre, noncontrolled study. Dogs were treated with a shampoo and lotion containing gluconolactone and other hydroxyl acids. Treatments were administered initially twice weekly for two weeks, then once weekly for two weeks and finally once monthly. Examinations were performed prior to and at 14 and 30 days of treatment to assess scaling, presence of other skin lesions and pruritus. In two dogs, pre- and 30 day post-treatment, skin biopsies were obtained. RESULTS: The extent and size of the scales were reduced by 60% and 75% after 14 and 30 days of treatment, respectively (P
Source: Veterinary Dermatology - Category: Veterinary Research Authors: Tags: Vet Dermatol Source Type: research

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Source: The British Journal of Dermatology - Category: Dermatology Authors: Tags: Br J Dermatol Source Type: research
CONCLUSIONS: Emulsion-A successfully generated the B-W SC in RHE for the first time. This method is suggested to be a useful tool for investigating the physiological significance of the B-W SC in vitro. Determination of Dsg1 content (Dsg1 pixels) in the SC obtained by tape-stripping from human skin can make it possible to study the effects of external stimulants, such as creams/ointments, including cosmetics, on the completeness of the B-W SC in situ without biopsy. This article is protected by copyright. All rights reserved. PMID: 31077338 [PubMed - as supplied by publisher]
Source: The British Journal of Dermatology - Category: Dermatology Authors: Tags: Br J Dermatol Source Type: research
Conclusions In this review, we collected all the information concerning the OMICs studies performed on HS patients aimed at unraveling the mechanisms at the basis of the disease or associated to clinical severity and/or the successful response to pharmacological treatment (including biological drugs). The general picture of the OMICs contribution in the context of HS is not so clear and/or rich of clinical useful information, since most of the studies focused only on one aspect (genome, transcriptome, or proteome) of the disease, enrolling small numbers of patients (this is quite limiting for the genetic studies) from di...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
Contributors : Hanqian Zhang ; Maja Ericsson ; Simone Westr öm ; Anders Vahlquist ; Marie Virtanen ; Hans TörmäSeries Type : Expression profiling by arrayOrganism : Homo sapiensAutosomal recessive congenital ichthyosis (ARCI) is a heterogeneous group of monogenic skin disorders caused by mutations in any of>10 different genes, many of which are involved in epidermal synthesis of ω-O-acylceramides (acylCer), an essential precursor of the corneocyte lipid envelope that is also dependent on transglutaminase-1 for normal skin barrier formation.  We hypothesized that inactivating TGM1 mutations, the...
Source: GEO: Gene Expression Omnibus - Category: Genetics & Stem Cells Tags: Expression profiling by array Homo sapiens Source Type: research
Publication date: May 2018 Source:Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, Volume 1864, Issue 5, Part A Author(s): Clare Rogerson, Paul Gissen Mutations in VPS33B and VIPAS39 cause the severe multisystem disorder Arthrogryposis, Renal dysfunction and Cholestasis (ARC) syndrome. Amongst other symptoms, patients with ARC syndrome suffer from severe ichthyosis. Roles for VPS33B and VIPAR have been reported in lysosome-related organelle biogenesis, integrin recycling, collagen homeostasis and maintenance of cell polarity. Mouse knockouts of Vps33b or Vipas39 are good models of ARC syndrome and develop...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - Category: Molecular Biology Source Type: research
Authors: Abstract Kaposi varicelliform eruption, also called eczema herpeticum, refers to a disseminated skin infection due to a virus that usually leads to localized vesicular eruptions, occurring in a patient with an underlying cutaneous disease. Although rare, it is potentially life-threatening disorder. Herpes simplex virus is considered the main causative agent. The most commonly reported cases occur in patients with atopic dermatitis. However, it has been described in association with other skin conditions such as pemphigus foliaceus, ichthyosis vulgaris, bullous pemphigoid, Darier disease, Grover disease, ...
Source: Herpes - Category: Infectious Diseases Tags: Book Source Type: research
Pollution is known to exacerbate asthma and atopic dermatitis (AD). Moreover, mice overexpressing xenobiotic receptors such as Aryl hydrocarbon receptor (AHR) or pregnane x receptor (PXR), in the epidermis develop AD-like symptoms. Furthermore, we have found increased expression of genes involved in xenobiotic metabolism in the skin of patients with AD but not with ichthyosis vulgaris (IV) by using RNA sequencing technology. To further investigate the possible link between enhanced metabolism of noxious molecules in AD and to validate data from our RNA sequencing analysis, we performed immunohistochemistry and real-time PC...
Source: Journal of Investigative Dermatology - Category: Dermatology Authors: Tags: Inflammation, Immunity and Infection Source Type: research
We present a 68-year-old human T-cell lymphotropic virus type 1 seropositive female with a mild peripheral blood atypical lymphocytosis who had indurated nodules on her hands of 2 years duration and a new scaly ichthyosiform eruption on her lower extremities. Histopathologic examination of the hand biopsy revealed coalescing nodules of large atypical noncerebriform lymphocytes with focal areas of epidermotropism. Phenotypically, the infiltrate was positive for β-F1, CD2, CD4, CD5, CD7, Foxp3, and CD25. In both biopsies, there was striking upregulation of TOX (thymocyte selection–associated high mobility group bo...
Source: The American Journal of Dermatopathology - Category: Pathology Tags: Original Study Source Type: research
Publication date: September–December 2015 Source:Arab Journal of Gastroenterology, Volume 16, Issues 3–4 Author(s): Engy A. Mogahed, Ahmed El-Hennawy, Rokaya El-Sayed, Hanaa El-Karaksy Chanarin–Dorfman syndrome, a “neutral lipid storage disease with ichthyosis,” is a multisystem inherited metabolic disorder associated with congenital ichthyosis and accumulation of lipid droplets in various types of cells. Case report A 3-year-old male presented to the Pediatric Hepatology Unit, Cairo University Children’s Hospital, Cairo, Egypt, with accidentally discovered hepatomegaly. He had ge...
Source: Arab Journal of Gastroenterology - Category: Gastroenterology Source Type: research
Shwachman–Diamond syndrome (SDS) is a recessive ribosomopathy, characterized by bone marrow failure and exocrine pancreatic insufficiency (ePI) often associated with neurodevelopmental and skeletal abnormalities. The aim of this report is to describe a SDS patient with early ichthyosis associated with dermal and epidermal intracellular lipid droplets (iLDs), hypoglycemia and later a distinctive clinical SDS phenotype. At 3 months of age, she had ichthyosis, growth retardation, and failure to thrive. She had not cytopenia. Ultrasonography (US) showed pancreatic diffuse high echogenicity. Subsequently fasting hypoketot...
Source: American Journal of Medical Genetics Part A - Category: Genetics & Stem Cells Authors: Tags: Original Article Source Type: research
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