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Palmoplantar blistering

MedGen UID:
870430
Concept ID:
C4024876
Finding
HPO: HP:0007446

Definition

A type of blistering that affects the skin of the palms of the hands and the soles of the feet. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Palmoplantar blistering

Conditions with this feature

Epidermolysis bullosa simplex 1C, localized
MedGen UID:
87016
Concept ID:
C0080333
Disease or Syndrome
Epidermolysis bullosa simplex (EBS) is characterized by fragility of the skin (and mucosal epithelia in some instances) that results in non-scarring blisters and erosions caused by minor mechanical trauma. EBS is distinguished from other types of epidermolysis bullosa (EB) or non-EB skin fragility syndromes by the location of the blistering in relation to the dermal-epidermal junction. In EBS, blistering occurs within basal keratinocytes. The severity of blistering ranges from limited to hands and feet to widespread involvement. Additional features can include hyperkeratosis of the palms and soles (keratoderma), nail dystrophy, milia, and hyper- and/or hypopigmentation. Rare EBS subtypes have been associated with additional clinical features including pyloric atresia, muscular dystrophy, cardiomyopathy, and/or nephropathy.
Epidermolysis bullosa with diaphragmatic hernia
MedGen UID:
346473
Concept ID:
C1856933
Disease or Syndrome
Epidermolysis bullosa simplex due to plakophilin deficiency
MedGen UID:
388032
Concept ID:
C1858302
Disease or Syndrome
Ectodermal dysplasia/skin fragility syndrome (EDSFS) is an autosomal recessive genodermatosis characterized by widespread skin fragility, alopecia, nail dystrophy, and focal keratoderma with painful fissures. Hypohidrosis and cheilitis are sometimes present (summary by Ersoy-Evans et al., 2006).
Palmoplantar keratoderma i, striate, focal, or diffuse
MedGen UID:
419717
Concept ID:
C2931122
Disease or Syndrome
Striate palmoplantar keratoderma belongs to a group of skin diseases in which there is thickening of the skin on the palms and soles. The striate form is characterized by longitudinal hyperkeratotic lesions extending the length of each finger to the palm, and hyperkeratotic lesions are restricted to regions of the body where pressure and abrasion are greatest (summary by Hunt et al., 2001). Patients with diffuse or focal forms of keratoderma associated with mutation in the DSG1 gene have also been reported (Keren et al., 2005; Milingou et al., 2006). Genetic Heterogeneity of Keratosis Palmoplantaris Striata Type II PPKS (PPKS2; 612908) is caused by mutation in the DSP gene (125647) on chromosome 6. Type III PPKS (PPKS3; 607654) is caused by mutation in the keratin-1 gene (KRT1; 139350) on chromosome 12q. For a general phenotypic description and a discussion of genetic heterogeneity of palmoplantar keratoderma (PPK), see epidermolytic PPK (144200). Nitoiu et al. (2014) reviewed desmosome biology in cardiocutaneous syndromes and inherited skin disease, including discussion of the involvement of the DSG1 and DSP genes.
Epidermolysis bullosa simplex, Dowling-Meara type, with severe palmoplantar keratoderma
MedGen UID:
864672
Concept ID:
C4016235
Disease or Syndrome
Epidermolysis bullosa simplex 2B, generalized intermediate
MedGen UID:
1794219
Concept ID:
C5562009
Disease or Syndrome
Generalized intermediate epidermolysis bullosa simplex-2B (EBS2B) is an autosomal dominant disorder of skin in which intraepidermal blistering occurs after minor mechanical trauma. Skin blistering is generalized, begins at birth, and is worsened by heat, humidity, and sweating. The tendency to blistering diminishes in adolescence, when it may become localized to hands and feet. Intermediate EBS has previously been known as the Koebner type (summary by Has et al., 2020). For a discussion of genetic heterogeneity of the subtypes of EBS, see EBS1A (131760). Reviews Has et al. (2020) reviewed characteristic features and molecular bases of the subtypes of epidermolysis bullosa, and provided a consensus reclassification of disorders with skin fragility.
Epidermolysis bullosa simplex 2C, localized
MedGen UID:
1794221
Concept ID:
C5562011
Disease or Syndrome
Epidermolysis bullosa simplex (EBS) is characterized by fragility of the skin (and mucosal epithelia in some instances) that results in non-scarring blisters and erosions caused by minor mechanical trauma. EBS is distinguished from other types of epidermolysis bullosa (EB) or non-EB skin fragility syndromes by the location of the blistering in relation to the dermal-epidermal junction. In EBS, blistering occurs within basal keratinocytes. The severity of blistering ranges from limited to hands and feet to widespread involvement. Additional features can include hyperkeratosis of the palms and soles (keratoderma), nail dystrophy, milia, and hyper- and/or hypopigmentation. Rare EBS subtypes have been associated with additional clinical features including pyloric atresia, muscular dystrophy, cardiomyopathy, and/or nephropathy.

Professional guidelines

PubMed

Ge M, Ji C, Li H, Huang H
DNA Cell Biol 2023 Oct;42(10):645-652. Epub 2023 Aug 10 doi: 10.1089/dna.2023.0154. PMID: 37566479
Crowley JJ, Pariser DM, Yamauchi PS
Postgrad Med 2021 Apr;133(3):330-344. Epub 2020 Oct 29 doi: 10.1080/00325481.2020.1831315. PMID: 33118424
Upitis JA, Krol A
J Cutan Med Surg 2002 May-Jun;6(3):214-7. Epub 2002 Apr 15 doi: 10.1007/s10227-001-0050-9. PMID: 11951129

Recent clinical studies

Etiology

Crowley JJ, Pariser DM, Yamauchi PS
Postgrad Med 2021 Apr;133(3):330-344. Epub 2020 Oct 29 doi: 10.1080/00325481.2020.1831315. PMID: 33118424
Weinberg RL, Coulombe PA, Polydefkis M, Caterina MJ
Br J Dermatol 2020 Mar;182(3):543-551. Epub 2019 Jul 24 doi: 10.1111/bjd.17880. PMID: 30883689
Wong A, Malvestiti AA, Hafner Mde F
Rev Assoc Med Bras (1992) 2016 Sep-Oct;62(5):468-73. doi: 10.1590/1806-9282.62.05.468. PMID: 27656858
Hamada T, Tsuruta D, Fukuda S, Ishii N, Teye K, Numata S, Dainichi T, Karashima T, Ohata C, Furumura M, Hashimoto T
Exp Dermatol 2013 Feb;22(2):83-7. Epub 2012 Oct 8 doi: 10.1111/exd.12021. PMID: 23039137
Brooke MA, Nitoiu D, Kelsell DP
J Pathol 2012 Jan;226(2):158-71. Epub 2011 Nov 14 doi: 10.1002/path.3027. PMID: 21989576

Diagnosis

Crowley JJ, Pariser DM, Yamauchi PS
Postgrad Med 2021 Apr;133(3):330-344. Epub 2020 Oct 29 doi: 10.1080/00325481.2020.1831315. PMID: 33118424
Kridin K, Ahmed AR
Front Immunol 2019;10:2466. Epub 2019 Oct 22 doi: 10.3389/fimmu.2019.02466. PMID: 31695695Free PMC Article
Jen M, Nallasamy S
Clin Dermatol 2016 Mar-Apr;34(2):242-75. Epub 2015 Dec 2 doi: 10.1016/j.clindermatol.2015.11.008. PMID: 26903188
Ventarola D, Bordone L, Silverberg N
Clin Dermatol 2015 May-Jun;33(3):340-6. Epub 2014 Dec 9 doi: 10.1016/j.clindermatol.2014.12.011. PMID: 25889136
Marovt M, El Shabrawi-Caelen L
Am J Dermatopathol 2015 Feb;37(2):e18-20. doi: 10.1097/DAD.0000000000000057. PMID: 25607328

Therapy

Lestido V, Rodríguez F, Rodríguez A, Pombo V, Barrios R, Pavani C
BMJ Open 2024 Apr 23;14(4):e081459. doi: 10.1136/bmjopen-2023-081459. PMID: 38657999Free PMC Article
Le HTT, Truong Van C, Nguyen Thi M, Al-Niaimi F
J Cosmet Laser Ther 2022 Jul 4;24(1-5):28-32. Epub 2022 Jul 14 doi: 10.1080/14764172.2022.2050761. PMID: 35833729
Obeid G, Do G, Kirby L, Hughes C, Sbidian E, Le Cleach L
Cochrane Database Syst Rev 2020 Jan 20;1(1):CD011628. doi: 10.1002/14651858.CD011628.pub2. PMID: 31958161Free PMC Article
Marovt M, El Shabrawi-Caelen L
Am J Dermatopathol 2015 Feb;37(2):e18-20. doi: 10.1097/DAD.0000000000000057. PMID: 25607328
Sehgal VN, Kumar S, Narayan S
Int J Dermatol 2001 Feb;40(2):130-2. doi: 10.1046/j.1365-4362.2001.01116.x. PMID: 11328395

Prognosis

Andrei D, Bremer J, Kramer D, Nijenhuis AM, van der Molen M, Diercks GFH, van den Akker PC, Vermeer MCSC, van der Meer P, Bolling MC
Exp Dermatol 2024 Mar;33(3):e15046. doi: 10.1111/exd.15046. PMID: 38509711
Duretz C, Antonicelli F, Muller C, Antonicelli N, Plee J, Viguier M, Bernard P
JAMA Dermatol 2019 Feb 1;155(2):216-220. doi: 10.1001/jamadermatol.2018.4084. PMID: 30484821Free PMC Article
Wong A, Malvestiti AA, Hafner Mde F
Rev Assoc Med Bras (1992) 2016 Sep-Oct;62(5):468-73. doi: 10.1590/1806-9282.62.05.468. PMID: 27656858
Marovt M, El Shabrawi-Caelen L
Am J Dermatopathol 2015 Feb;37(2):e18-20. doi: 10.1097/DAD.0000000000000057. PMID: 25607328
Chamcheu JC, Siddiqui IA, Syed DN, Adhami VM, Liovic M, Mukhtar H
Arch Biochem Biophys 2011 Apr 15;508(2):123-37. Epub 2010 Dec 19 doi: 10.1016/j.abb.2010.12.019. PMID: 21176769Free PMC Article

Clinical prediction guides

Putra PB, Radiono S, Danarti R
Dermatol Online J 2021 Feb 15;27(2) PMID: 33818988
Duretz C, Antonicelli F, Muller C, Antonicelli N, Plee J, Viguier M, Bernard P
JAMA Dermatol 2019 Feb 1;155(2):216-220. doi: 10.1001/jamadermatol.2018.4084. PMID: 30484821Free PMC Article
Marovt M, El Shabrawi-Caelen L
Am J Dermatopathol 2015 Feb;37(2):e18-20. doi: 10.1097/DAD.0000000000000057. PMID: 25607328
Smith F
Am J Clin Dermatol 2003;4(5):347-64. doi: 10.2165/00128071-200304050-00005. PMID: 12688839
Korge BP, Krieg T
J Mol Med (Berl) 1996 Feb;74(2):59-70. doi: 10.1007/BF00196781. PMID: 8820401

Recent systematic reviews

Obeid G, Do G, Kirby L, Hughes C, Sbidian E, Le Cleach L
Cochrane Database Syst Rev 2020 Jan 20;1(1):CD011628. doi: 10.1002/14651858.CD011628.pub2. PMID: 31958161Free PMC Article
Kridin K, Ahmed AR
Front Immunol 2019;10:2466. Epub 2019 Oct 22 doi: 10.3389/fimmu.2019.02466. PMID: 31695695Free PMC Article

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