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unna thost syndrome 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, as the former exhibits autosomal dominant inheritance and hyperhidrosis is frequently present.


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Additional comments :
(Diffuse Palmoplantar Keratoderma, Tylosis)

Articles



PubmedA unique pattern of dyskeratosis characterizes epidermolytic hyperkeratosis and epidermolytic palmoplantar keratoderma.
Authors:  Bergman R, Khamaysi Z, Sprecher E
Journal: Am J Dermatopathol

Pubmed[Papillon-Lefevre syndrome: a case report]
Authors:  Lin CT, Ai YH, Chen Y
Journal: Hua Xi Kou Qiang Yi Xue Za Zhi

PubmedIchthyosis cribriformis: A new entity?
Authors:  Larangeira de Almeida H, Happle R, Blume-Peytavi U, de Castro LA
Journal: J Am Acad Dermatol

PubmedGenetics of gastroesophageal cancer: paradigms, paradoxes, and prognostic utility.
Authors:  Robertson EV, Jankowski JA
Journal: Am J Gastroenterol

PubmedGreither's disease.
Authors:  Athanikar SB, Inamadar AC, Palit A, Sampagavi VV, Deshmukh NS
Journal: Indian J Dermatol Venereol Leprol

PubmedCapecitabine-induced diffuse palmoplantar keratoderma: is it a sequential event of hand-foot syndrome?
Authors:  Do JE, Kim YC
Journal: Clin Exp Dermatol

Pubmed[Isolated pandysautonomia: a rare cause of palmoplantar keratodermia]
Authors:  Augey F, Badet F, Diot E, Daumont A
Journal: Ann Dermatol Venereol

PubmedKeratoderma hereditarium mutilans (Vohwinkel syndrome) in three siblings.
Authors:  ul Bari A
Journal: Dermatol Online J

Pubmed[Autosomal dominant punctate palmoplantar keratoderma (Buschke-Fischer-Brauer disease)]
Authors:  Kluger N, Augias D, Guillot B
Journal: Ann Dermatol Venereol

Pubmed[Pathological features and gene mutation analysis in two pedigrees of diffuse palmoplantar keratoderma]
Authors:  Yin XZ, Zhang BR, Ding MP, Zhang H, Xia K, Hu ZM
Journal: Yi Chuan

PubmedMutation of keratin 9 (R163W) in a family with epidermolytic palmoplantar keratoderma and knuckle pads.
Authors:  Chiu HC, Jee SH, Sheen YS, Chu CY, Lin PJ, Liaw SH
Journal: J Dermatol Sci

PubmedHereditary diffuse palmoplantar keratodermas in Slovenia: epidemiologic foci in remote rural areas.
Authors:  Miljkovi? J, Kansky A, Vidmar G
Journal: Wien Klin Wochenschr

PubmedVenlafaxine-associated psoriasiform palmoplantar keratoderma and subungual hyperkeratosis.
Authors:  Dalle S, Becuwe C, Balme B, Thomas L
Journal: Br J Dermatol

PubmedDown-regulation of the cytoglobin gene, located on 17q25, in tylosis with oesophageal cancer (TOC): evidence for trans-allele repression.
Authors:  McRonald FE, Liloglou T, Xinarianos G, Hill L, Rowbottom L, Langan JE, Ellis A, Shaw JM, Field JK, Risk JM
Journal: Hum Mol Genet

PubmedA 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.
Authors:  Richardson ES, Lee JB, Hyde PH, Richard G
Journal: J Invest Dermatol

PubmedDiagnosis of epidermolytic palmoplantar keratoderma in a very early stage by gene analysis.
Authors:  Shimazu K, Tsunemi Y, Hattori N, Adachi M, Imakado S, Saeki H, Komine M, Tamaki K
Journal: J Dermatol

Pubmed[Hotspot of the mutations of keratin 9 gene in a diffuse palmoplantar keratoderma family]
Authors:  Sun X, Yin XZ, Wu LQ, Shi XL, Hu ZM, Liu XP, Pan Q, Dai HP, Xia K, Xia JH
Journal: Zhong Nan Da Xue Xue Bao Yi Xue Ban

PubmedTyrosinaemia type II with diffuse plantar keratoderma and self-mutilation.
Authors:  Madan V, Gupta U
Journal: Clin Exp Dermatol

PubmedThe common KRT9 gene mutation in a Japanese patient with epidermolytic palmoplantar keratoderma and knuckle pad-like keratoses.
Authors:  Hamada T, Ishii N, Karashima T, Kawano Y, Yasumoto S, Hashimoto T
Journal: J Dermatol

PubmedInfrequent mutation of the human envoplakin gene is closely linked to the tylosis oesophageal cancer locus in sporadic oesophageal squamous cell carcinomas.
Authors:  Iwaya T, Maesawa C, Kimura T, Ogasawara S, Ikeda K, Kimura Y, Noda Y, Ishida K, Sato N, Saito K, Masuda T
Journal: Oncol Rep

PubmedPalmoplantar keratodermas.
Authors:  Itin PH, Fistarol SK
Journal: Clin Dermatol

PubmedAn investigation of the tylosis with oesophageal cancer (TOC) locus in Iranian patients with oesophageal squamous cell carcinoma.
Authors:  Shahabi M, Noori Daloii MR, Langan JE, Rowbottom L, Jahanzad E, Khoshbin E, Taghikhani M, Field JK, Risk JM
Journal: Int J Oncol

PubmedMultiple myeloma in a patient with systemic lupus erythematosus, myasthenia gravis and non-familial diffuse palmoplantar keratoderma.
Authors:  Urba?ska-Ry? H, Robak E, Kordek R, Bartkowiak J, Rieske P, Wo?niacka A, Smolewski P, Robak T
Journal: Leuk Lymphoma

PubmedNovel microsatellite markers and single nucleotide polymorphisms refine the tylosis with oesophageal cancer (TOC) minimal region on 17q25 to 42.5 kb: sequencing does not identify the causative gene.
Authors:  Langan JE, Cole CG, Huckle EJ, Byrne S, McRonald FE, Rowbottom L, Ellis A, Shaw JM, Leigh IM, Kelsell DP, Dunham I, Field JK, Risk JM
Journal: Hum Genet

PubmedAn unusual case of palmoplantar keratoderma.
Authors:  Devos SA, Delescluse J
Journal: J Eur Acad Dermatol Venereol

PubmedCarcinoma of stomach in a patient with familial tylosis.
Authors:  Wagle PK, Shetty TS, Darbari A, Tapia AA, Katrak MP, Joshi RM
Journal: Indian J Gastroenterol

PubmedA keratin 9 Gene mutation (Asn160Ser) in a Japanese patient with epidermolytic palmoplantar keratoderma.
Authors:  Tsunemi Y, Hattori N, Saeki H, Adachi M, Komine M, Nakagawa H, Tamaki K
Journal: J Dermatol

PubmedMal de Meleda (MDM) caused by mutations in the gene for SLURP-1 in patients from Germany, Turkey, Palestine, and the United Arab Emirates.
Authors:  Eckl KM, Stevens HP, Lestringant GG, Westenberger-Treumann M, Traupe H, Hinz B, Frossard PM, Stadler R, Leigh IM, Nürnberg P, Reis A, Hennies HC
Journal: Hum Genet

PubmedCharacterization of a 500 kb region on 17q25 and the exclusion of candidate genes as the familial Tylosis Oesophageal Cancer (TOC) locus.
Authors:  Risk JM, Evans KE, Jones J, Langan JE, Rowbottom L, McRonald FE, Mills HS, Ellis A, Shaw JM, Leigh IM, Kelsell DP, Field JK
Journal: Oncogene

PubmedEpidermolytic palmoplantar keratoderma of Vörner: re-evaluation of Vörner's original family and identification of a novel keratin 9 mutation.
Authors:  Küster W, Reis A, Hennies HC
Journal: Arch Dermatol Res

PubmedDiagnosis and confirmation of epidermolytic palmoplantar keratoderma by the identification of mutations in keratin 9 using denaturing high-performance liquid chromatography.
Authors:  Rugg EL, Common JE, Wilgoss A, Stevens HP, Buchan J, Leigh IM, Kelsell DP
Journal: Br J Dermatol

PubmedNon-small-cell lung cancer with nonfamilial diffuse palmoplantar keratoderma.
Authors:  Engin H, Akdogan A, Altundag O, Kars A, Güler N
Journal: J Exp Clin Cancer Res

PubmedCompound heterozygosity for non-sense and mis-sense mutations in desmoplakin underlies skin fragility/woolly hair syndrome.
Authors:  Whittock NV, Wan H, Morley SM, Garzon MC, Kristal L, Hyde P, McLean WH, Pulkkinen L, Uitto J, Christiano AM, Eady RA, McGrath JA
Journal: J Invest Dermatol

PubmedConnexin disorders of the skin.
Authors:  Richard G
Journal: Adv Dermatol

PubmedDysphagia in a patient with palmoplantar keratoderma.
Authors:  Morgan R
Journal: Postgrad Med J

PubmedTransgrediens et progrediens palmoplantar keratoderma (Greither's disease) with particular histopathologic findings.
Authors:  Grilli R, Aguilar A, Escalonilla P, Soriano L, Fariña C, Martín L, Requena L
Journal: Cutis

PubmedThe tylosis esophageal cancer (TOC) locus: more than just a familial cancer gene.
Authors:  Risk JM, Mills HS, Garde J, Dunn JR, Evans KE, Hollstein M, Field JK
Journal: Dis Esophagus

PubmedEpidermolytic hyperkeratosis with polycyclic psoriasiform plaques resulting from a mutation in the keratin 1 gene.
Authors:  Michael EJ, Schneiderman P, Grossman ME, Christiano AM
Journal: Exp Dermatol

PubmedFine genetic mapping of diffuse non-epidermolytic palmoplantar keratoderma to chromosome 12q11-q13: exclusion of the mapped type II keratins.
Authors:  Kelsell DP, Stevens HP, Purkis PE, Talas U, Rustin MH, Leigh IM
Journal: Exp Dermatol

PubmedEnvoplakin, a possible candidate gene for focal NEPPK/esophageal cancer (TOC): the integration of genetic and physical maps of the TOC region on 17q25.
Authors:  Risk JM, Ruhrberg C, Hennies H, Mills HS, Di Colandrea T, Evans KE, Ellis A, Watt FM, Bishop DT, Spurr NK, Stevens HP, Leigh IM, Reis A, Kelsell DP, Field JK
Journal: Genomics

PubmedTo B or not to B: is tylosis B truly benign? Two North American genealogies.
Authors:  Maillefer RH, Greydanus MP
Journal: Am J Gastroenterol

PubmedA case of Unna-Thost disease accompanied by Epidermophyton floccosum infection.
Authors:  Maruyama R, Katoh T, Nishioka K
Journal: J Dermatol

PubmedPalmoplantar keratoderma and leukokeratosis anogenitalis: the second case of a new disease.
Authors:  Lautenschlager S, Pittelkow MR
Journal: Dermatology

PubmedLoss of heterozygosity in sporadic oesophageal tumors in the tylosis oesophageal cancer (TOC) gene region of chromosome 17q.
Authors:  von Brevern M, Hollstein MC, Risk JM, Garde J, Bennett WP, Harris CC, Muehlbauer KR, Field JK
Journal: Oncogene

PubmedTylosis esophageal cancer locus on chromosome 17q25.1 is commonly deleted in sporadic human esophageal cancer.
Authors:  Iwaya T, Maesawa C, Ogasawara S, Tamura G
Journal: Gastroenterology

PubmedPalmoplantar keratoderma associated with congenital heart disease.
Authors:  Hoeger PH, Yates RW, Harper JI
Journal: Br J Dermatol

Pubmed[The Clark-Howel-Evans-McConnell syndrome. Observations in one family over 5 generations]
Authors:  Simon M, Hagedorn M
Journal: Hautarzt

PubmedStructural organization of cornified cell envelopes and alterations in inherited skin disorders.
Authors:  Ishida-Yamamoto A, Iizuka H
Journal: Exp Dermatol

PubmedHereditary palmoplantar keratodermas in South India.
Authors:  Gulati S, Thappa DM, Garg BR
Journal: J Dermatol

PubmedThe inherited palmoplantar keratodermas.
Authors:  Ratnavel RC, Griffiths WA
Journal: Br J Dermatol

PubmedA novel nonepidermolytic palmoplantar keratoderma: a clinical and histopathologic study of six cases.
Authors:  Magro CM, Baden LA, Crowson AN, Bowden PE, Baden HP
Journal: J Am Acad Dermatol

PubmedFrontiers in keratodermas: pushing the envelope.
Authors:  Christiano AM
Journal: Trends Genet

PubmedDermatomyositis with a pityriasis rubra pilaris-like eruption: a little-known distinctive cutaneous manifestation of dermatomyositis.
Authors:  Requena L, Grilli R, Soriano L, Escalonilla P, Fariña C, Martín L
Journal: Br J Dermatol

PubmedHaber's syndrome.
Authors:  McCormack CJ, Cowen P
Journal: Australas J Dermatol

PubmedOral tylosis: a re-appraisal.
Authors:  Field EA, Ellis A, Friedmann PS, Leigh IM, Field JK
Journal: Oral Oncol

PubmedChromosomal localisation of the human envoplakin gene (EVPL) to the region of the tylosis oesophageal cancer gene (TOCG) on 17q25.
Authors:  Ruhrberg C, Williamson JA, Sheer D, Watt FM
Journal: Genomics

PubmedHereditary palmoplantar keratoderma with deafness.
Authors:  Fitzgerald DA, Verbov JL
Journal: Br J Dermatol

Pubmed[Vörner keratosis palmoplantaris diffusa. Clinical, formal genetic and molecular biology studies of 22 families]
Authors:  Küster W, Zehender D, Mensing H, Hennies HC, Reis A
Journal: Hautarzt

PubmedDiffuse palmoplantar keratoderma associated with acrocyanosis and livedo reticularis. Two sporadic cases.
Authors:  Ohtake N, Sou K, Tsukamoto K, Furue M, Tamaki K
Journal: Acta Derm Venereol

PubmedKeratoderma hereditarium mutilans (Vohwinkel's syndrome) associated with congenital deaf-mutism.
Authors:  Peris K, Salvati EF, Torlone G, Chimenti S
Journal: Br J Dermatol

Pubmed[Osteopoikilosis--skin and joint manifestations]
Authors:  Colla F, Brühlmann P, Panizzon R, Michel BA
Journal: Z Rheumatol

PubmedDiffuse plane xanthomatosis and acquired palmoplantar keratoderma in association with myeloma.
Authors:  Smith CH, Barker JN, Hay RJ
Journal: Br J Dermatol

PubmedMiscellaneous genodermatoses: Beckwith-Wiedemann syndrome, Birt-Hogg-Dube syndrome, familial atypical multiple mole melanoma syndrome, hereditary tylosis, incontinentia pigmenti, and supernumerary nipples.
Authors:  Cohen PR, Kurzrock R
Journal: Dermatol Clin

PubmedVerrucous carcinoma in Unna-Thost hyperkeratosis of the palms and soles.
Authors:  Rogozi?ski TT, Schwartz RA, Towpik E
Journal: J Am Acad Dermatol

PubmedTylosis oesophageal cancer mapped.
Authors:  Risk JM, Field EA, Field JK, Whittaker J, Fryer A, Ellis A, Shaw JM, Friedmann PS, Bishop DT, Bodmer J
Journal: Nat Genet

PubmedA mutation in the V1 end domain of keratin 1 in non-epidermolytic palmar-plantar keratoderma.
Authors:  Kimonis V, DiGiovanna JJ, Yang JM, Doyle SZ, Bale SJ, Compton JG
Journal: J Invest Dermatol

PubmedKeratin gene mutations in human skin disease.
Authors:  Stevens HP, Rustin MH
Journal: Postgrad Med J

PubmedThe gene for diffuse palmoplantar keratoderma of the type found in northern Sweden is localized to chromosome 12q11-q13.
Authors:  Lind L, Lundström A, Hofer PA, Holmgren G
Journal: Hum Mol Genet

PubmedA comparative histopathological examination of biopsies from patients with either the dominant or the Gamborg Nielsen type of hereditary palmoplantar keratoderma.
Authors:  Gamborg Nielsen P, Mölne L
Journal: Dermatology

PubmedTylosis associated with carcinoma of the oesophagus and oral leukoplakia in a large Liverpool family--a review of six generations.
Authors:  Ellis A, Field JK, Field EA, Friedmann PS, Fryer A, Howard P, Leigh IM, Risk J, Shaw JM, Whittaker J
Journal: Eur J Cancer B Oral Oncol

PubmedThe dominant form of hereditary palmoplantar keratoderma in the northernmost county of Sweden (Norrbotten).
Authors:  Gamborg Nielsen P, Hofer PA, Lagerholm B
Journal: Dermatology

Pubmed[Mapping of the dominant gene of hyperkeratosis palmaris et plantaris in man]
Authors:  Rogaev EI, Korova?tseva GI, Ginter EK, Prytkov AN, Maksudova KhA
Journal: Genetika

PubmedNatural history of the Naegeli-Franceschetti-Jadassohn syndrome and further delineation of its clinical manifestations.
Authors:  Itin PH, Lautenschlager S, Meyer R, Mevorah B, Rufli T
Journal: J Am Acad Dermatol

PubmedHuman papillomavirus in squamous cell carcinoma of the oesophagus associated with tylosis.
Authors:  Ashworth MT, McDicken IW, Southern SA, Nash JR
Journal: J Clin Pathol

Pubmed[Olmsted syndrome. Successful therapy by treatment with etretinate]
Authors:  Hausser I, Frantzmann Y, Anton-Lamprecht I, Estes S, Frosch PJ
Journal: Hautarzt

PubmedNonfamilial diffuse palmoplantar keratoderma associated with bronchial carcinoma.
Authors:  Khanna SK, Agnone FA, Leibowitz AI, Raschke RA, Trehan M
Journal: J Am Acad Dermatol

PubmedKeratosis extremitatum (Greither's disease): clinical features, histology, ultrastructure.
Authors:  Flückiger R, Itin PH
Journal: Dermatology

PubmedPalmoplantar keratoderma of Unna-Thost: response to biotin in one family.
Authors:  Menni S, Saleh F, Piccinno R, Corbetta C, Melotti D
Journal: Clin Exp Dermatol

PubmedAcitretin in the treatment of mal de Meleda.
Authors:  van de Kerkhof PC, van Dooren-Greebe RJ, Steijlen PM
Journal: Br J Dermatol

PubmedIsolated cases of palmoplantar keratoderma, Unna-Thost type.
Authors:  Kansky A, Stanimirovi? A, Basta-Juzbasic A
Journal: Cutis

PubmedPalmoplantar keratoderma with sclerodactyly (Huriez syndrome).
Authors:  Patrizi A, Di Lernia V, Patrone P
Journal: J Am Acad Dermatol

PubmedSpiny keratoderma of the palms and soles.
Authors:  Osman Y, Daly TJ, Don PC
Journal: J Am Acad Dermatol

PubmedIndication for the identity of palmoplantar keratoderma type Unna-Thost with type Vörner. Thost's family revisited 110 years later.
Authors:  Küster W, Becker A
Journal: Acta Derm Venereol

PubmedPalmoplantar keratoderma in a noncutaneous T-cell lymphoma.
Authors:  Trattner A, Katzenelson V, Sandbank M
Journal: Int J Dermatol

PubmedHereditary epidermolytic palmoplantar keratoderma with knuckle pad-like lesions over the finger joints.
Authors:  Nogita T, Nakagawa H, Ishibashi Y
Journal: Br J Dermatol

PubmedAbnormalities of differentiation and maturation in the oesophageal squamous epithelium of patients with tylosis: morphological features.
Authors:  Ashworth MT, Nash JR, Ellis A, Day DW
Journal: Histopathology

PubmedHereditary epidermolytic palmo-plantar keratoderma (Vörner type)--report of a family and review of the literature.
Authors:  Requena L, Schoendorff C, Sanchez Yus E
Journal: Clin Exp Dermatol

PubmedErythroderma, palmoplantar keratoderma and profound failure to thrive in an infant.
Authors:  Judge MR, Shield JP, Cant A, Strobel S, Levin M, Reiser J, Harper JI
Journal: Br J Dermatol

Pubmed[Pheochromocytoma and palmoplantar keratoderma]
Authors:  Claudy AL, Segault D, Rousset H, Verneyre H
Journal: Ann Dermatol Venereol

PubmedMal de Meleda-like palmoplantar keratoderma.
Authors:  Iio T, Shiraishi S, Sayama K, Miki Y
Journal: J Dermatol

PubmedCongenital heart disease in tylosis: case report.
Authors:  Wong ML, Tay JS
Journal: J Singapore Paediatr Soc

PubmedEpidermal growth factor receptors in genetically induced hyperproliferative skin disorders.
Authors:  Nanney LB, King LE, Dale BA
Journal: Pediatr Dermatol

PubmedAn experimental study on the healing process after excision of the articular disc of the mandible--effect of short-term intermaxillary fixation.
Authors:  Shimahara M, Ono K, Hashiguchi N, Senda J, Shimamura T, Honke T, Higashisawa S
Journal: Bull Osaka Med Coll

PubmedPathohistological study on changes observed in the temporomandibular joint after experimental mutilation and shortening of the mandibular process with special reference to the changes under abnormal mandibular movement.
Authors:  Shimahara M, Ono K, Hashiguchi N
Journal: Bull Osaka Med Coll

PubmedUnusual cutaneous manifestations of anhidrotic ectodermal dysplasia--a case report.
Authors:  Shah KC, Umrigar DD
Journal: J Dermatol

PubmedCharacterization of Candida albicans epidermolytic proteases and their role in yeast-cell adherence to keratinocytes.
Authors:  el-Maghrabi EA, Dixon DM, Burnett JW
Journal: Clin Exp Dermatol

PubmedHereditary palmoplantar keratosis of the Gamborg Nielsen type. Clinical and ultrastructural characteristics of a new type of autosomal recessive palmoplantar keratosis.
Authors:  Kastl I, Anton-Lamprecht I, Gamborg Nielsen P
Journal: Arch Dermatol Res

PubmedPalmoplantar hyperkeratosis with short stature, facial dysmorphism, and hypodontia--a new syndrome?: case report.
Authors:  Seow WK
Journal: Pediatr Dent

PubmedDiffuse palmoplantar keratoderma associated with acrocyanosis. A family study.
Authors:  Nielsen PG
Journal: Acta Derm Venereol

Pubmed[Keratosis palmoplantaris diffuse circumscripta (Thost-Unna) with micropenis]
Authors:  Kurz KH, Steigleder GK
Journal: Z Hautkr





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