Diffuse Palmoplantar Keratoderma (Tylosis)

An autosomal dominant disorder characterized by a widely distributed, well-demarcated hyperkeratosis of the palms and soles. There is more than one genotypically distinct form, each of which is clinically similar but histologically distinguishable. Diffuse palmoplantar keratoderma is distinct from palmoplantar keratoderma (KERATODERMA, PALMOPLANTAR), as the former exhibits autosomal dominant inheritance and hyperhidrosis is frequently present.

Also Known As:

Tylosis; Keratoderma, Palmoplantar, Diffuse; Keratosis Palmaris et Plantaris Familiaris; Syndrome, Thost-Unna; Syndrome, Unna-Thost; Thost Unna Syndrome; Unna Thost Syndrome; Thost-Unna Syndrome; Unna-Thost Syndrome

Networked: 35 relevant articles (1 outcomes, 0 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Neoplasms (Cancer)
2.	Esophageal Achalasia (Megaesophagus)
3.	Pathologic Constriction (Stenosis)
4.	Barrett Esophagus (Barrett's Esophagus)
5.	Plummer-Vinson Syndrome (Syndrome, Plummer-Vinson)

Experts

1.	Kelsell, David P: 3 articles (01/2017 - 06/2010)
2.	McRonald, Fiona E: 3 articles (01/2012 - 04/2006)
3.	Risk, Janet M: 3 articles (01/2012 - 04/2006)
4.	Hosur, Vishnu: 2 articles (11/2017 - 08/2017)
5.	Shultz, Leonard D: 2 articles (11/2017 - 08/2017)
6.	Brooke, Matthew A: 2 articles (01/2017 - 08/2014)
7.	Ishida-Yamamoto, Akemi: 2 articles (01/2017 - 08/2014)
8.	Field, John K: 2 articles (07/2006 - 04/2006)
9.	Liloglou, Triantafillos: 2 articles (07/2006 - 04/2006)
10.	Xinarianos, George: 2 articles (07/2006 - 04/2006)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Diffuse Palmoplantar Keratoderma:

1.	FertilizersIBA 03/03/2022 - "The efficacy of the synergistic application of alkaline fertilizer and biocontrol fungi was not only reducing Foc Tr4 pathogen colonization and distribution in banana plants, but also preventing tylosis formation in vascular vessel effectively. "
2.	CausticsIBA 04/01/2001 - "The surveillance of patients, especially those with tylosis or caustic ingestion, has been recommended. " 02/01/1975 - "A case of tylosis following corrosive stricture of the oesophagus in a male of 26 years is recorded. " 02/01/1975 - "Tylosis following post-corrosive stricture of the oesophagus." 12/01/2016 - "These high-risk groups include patients after curative treatment for head and neck cancer, previous endoscopic resection of ESCC, caustic injury, and patients with tylosis or achalasia. " 10/01/2019 - "This review article summarizes current diagnostic, management, and therapeutic practices of premalignant conditions of the esophagus including Barrett's esophagus, tylosis, granular cell tumors, achalasia, and the ingestion of caustic substances. "
3.	CytoglobinIBA 01/01/2012 - "Cytoglobin is a candidate for the tylosis with oesophageal cancer gene, and its expression is strongly down-regulated in non-cancerous oesophageal biopsies from patients with TOC compared with normal biopsies. " 07/01/2006 - "One candidate tumour-suppressor gene is cytoglobin (CYGB), which is contained entirely within the 42.5 kb tylosis with oesophageal cancer (TOC) minimal region. " 04/15/2006 - "Down-regulation of the cytoglobin gene, located on 17q25, in tylosis with oesophageal cancer (TOC): evidence for trans-allele repression."
4.	AmphiregulinIBA 08/15/2017 - "We generated a mouse model of human tylosis and show that GOF mutations in RHBDF2 cause tylosis by enhancing the amount of amphiregulin (AREG) secretion. " 08/15/2017 - "Genetic deletion of amphiregulin restores the normal skin phenotype in a mouse model of the human skin disease tylosis." 11/07/2017 - "The curly bare mouse model of tylosis, carrying a GOF mutation in the Rhbdf2 gene (Rhbdf2 cub ), presents with epidermal hyperplasia and shows accelerated cutaneous wound-healing phenotype through enhanced secretion of the epidermal growth factor receptor family ligand amphiregulin. "
5.	Keratin-1IBA 09/01/2009 - "Mutation L437P in the 2B domain of keratin 1 causes diffuse palmoplantar keratoderma in a Chinese pedigree." 01/01/2006 - "A novel mutation and large size polymorphism affecting the V2 domain of keratin 1 in an African-American family with severe, diffuse palmoplantar keratoderma of the ichthyosis hystrix Curth-Macklin type."
6.	LyeIBA 12/01/1989 - "These conditions include achalasia, Barrett's esophagus, chronic atrophic gastritis with intestinal metaplasia, familial polyposis coli, gastric polyps, lye stricture, Plummer-Vinson syndrome, and tylosis. " 03/01/2021 - "This makes early detection of the utmost importance, and certain patients have been shown to have the benefit of screening/surveillance endoscopies, including those with Barrett's esophagus, lye-induced/caustic strictures, tylosis, and Peutz-Jeghers syndrome. "
7.	envoplakinIBA 11/01/1996 - "Chromosomal localisation of the human envoplakin gene (EVPL) to the region of the tylosis oesophageal cancer gene (TOCG) on 17q25." 04/01/2005 - "Infrequent mutation of the human envoplakin gene is closely linked to the tylosis oesophageal cancer locus in sporadic oesophageal squamous cell carcinomas."
8.	ErbB Receptors (EGF Receptor)IBA 11/07/2017 - "The curly bare mouse model of tylosis, carrying a GOF mutation in the Rhbdf2 gene (Rhbdf2 cub ), presents with epidermal hyperplasia and shows accelerated cutaneous wound-healing phenotype through enhanced secretion of the epidermal growth factor receptor family ligand amphiregulin. "
9.	Keratin-9 (Keratin 9)IBA 10/01/2005 - "The mutation G485A found in keratin 9 gene is the disease-causing mutation in the diffuse palmoplantar keratoderma family." 10/01/2005 - "[Hotspot of the mutations of keratin 9 gene in a diffuse palmoplantar keratoderma family]."
10.	Type II Keratins (Type II Keratin)IBA 01/01/2017 - "Gain-of-function mutations in iRHOM2 underlie Tylosis with oesophageal cancer (TOC), characterized by palmoplantar thickening, upregulate K16 with robust downregulation of its type II keratin binding partner, K6. "

Therapies and Procedures

1.	Slit Lamp 07/01/2016 - "The following elements were found upon the slit lamp biomicroscopy: Eyelids - +4 palpebral edema (pseudoptosis), Dennie-Morgan fold and Herthoge's sign were both present, tylosis; Conjunctiva - hyperaemia, cobblestone appearance of the tarsal papillae in both eyes, +2 chemosis; Cornea - corneal edema with a 8 mm × 4 mm epithelial defect in the inferior part of the cornea, covered partially by the lied, that stained positive with fluorescein dyes. "
2.	Transjugular Intrahepatic Portasystemic Shunt 01/01/2015 - "Relative movement of plant cells relative to each other is expressed during fiber elongation, growth of arms of branched sclereids, intrusive growth of the tips of fusiform initials in the cambium, the increase in diameter of vessel members, growth in length of vessel-member elements in the secondary xylem of the few monocotyledons that express secondary growth, growth of laticifers, formation of tylosis, dilatation in the bark via parenchyma cell expansion, and growth of pollen tubes in the style. "
3.	Therapeutics 11/01/2021 - "Second-line antitubercular therapy with ethionamide and pyrazinamide causing pellagroid dermatitis presenting as diffuse palmoplantar keratoderma."