U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Palmoplantar keratoderma i, striate, focal, or diffuse(SPPK1; PPKS1; KPPS1)

MedGen UID:
419717
Concept ID:
C2931122
Disease or Syndrome
Synonyms: Keratoderma, palmoplantar striate form 1; KERATODERMA, PALMOPLANTAR, STRIATE FORM I; KERATOSIS PALMOPLANTARIS STRIATA I; Keratosis Palmoplantaris Striata I, AD; PALMOPLANTAR KERATODERMA I, FOCAL; PALMOPLANTAR KERATODERMA I, STRIATE OR DIFFUSE; Striate palmoplantar keratoderma 1; STRIATE PALMOPLANTAR KERATODERMA I
 
Gene (location): DSG1 (18q12.1)
 
Monarch Initiative: MONDO:0007859
OMIM®: 148700

Definition

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. [from OMIM]

Clinical features

From HPO
Palmoplantar keratosis
MedGen UID:
44017
Concept ID:
C0022596
Disease or Syndrome
Abnormal thickening of the skin localized to the palm of the hand and the sole of the foot.
Palmoplantar keratoderma
MedGen UID:
1635750
Concept ID:
C4551675
Disease or Syndrome
Abnormal thickening of the skin of the palms of the hands and the soles of the feet.
Hyperhidrosis
MedGen UID:
5690
Concept ID:
C0020458
Finding
Abnormal excessive perspiration (sweating) despite the lack of appropriate stimuli like hot and humid weather.
Onycholysis
MedGen UID:
39324
Concept ID:
C0085661
Disease or Syndrome
Detachment of the nail from the nail bed.
Nail dystrophy
MedGen UID:
66368
Concept ID:
C0221260
Disease or Syndrome
Onychodystrophy (nail dystrophy) refers to nail changes apart from changes of the color (nail dyschromia) and involves partial or complete disruption of the various keratinous layers of the nail plate.
Epidermal acanthosis
MedGen UID:
65136
Concept ID:
C0221270
Finding
Diffuse hypertrophy or thickening of the stratum spinosum of the epidermis (prickle cell layer of the skin).
Yellow nails
MedGen UID:
369197
Concept ID:
C1768507
Sign or Symptom
Yellowish discoloration of the nails.
Hypergranulosis
MedGen UID:
481177
Concept ID:
C3279547
Finding
Hypergranulosis is an increased thickness of the stratum granulosum.
Orthokeratotic hyperkeratosis
MedGen UID:
811321
Concept ID:
C3670629
Disease or Syndrome
A form of hyperkeratosis characterized by thickening of the cornified layer without retained nuclei.
Streaks of hyperkeratosis along each finger onto the palm
MedGen UID:
870416
Concept ID:
C4024861
Finding
Palmoplantar blistering
MedGen UID:
870430
Concept ID:
C4024876
Finding
A type of blistering that affects the skin of the palms of the hands and the soles of the feet.

Professional guidelines

PubMed

Rudnicka L, Arenbergerova M, Grimalt R, Ioannides D, Katoulis AC, Lazaridou E, Olszewska M, Ovcharenko YS, Piraccini BM, Prohic A, Rakowska A, Reygagne P, Richard MA, Soares RO, Starace M, Vañó-Galvan S, Waskiel-Burnat A
J Eur Acad Dermatol Venereol 2024 Apr;38(4):687-694. Epub 2024 Jan 2 doi: 10.1111/jdv.19768. PMID: 38169088
Attaway AH, Scheraga RG, Bhimraj A, Biehl M, Hatipoğlu U
BMJ 2021 Mar 10;372:n436. doi: 10.1136/bmj.n436. PMID: 33692022
Rosenfeld RM, Schwartz SR, Cannon CR, Roland PS, Simon GR, Kumar KA, Huang WW, Haskell HW, Robertson PJ
Otolaryngol Head Neck Surg 2014 Feb;150(1 Suppl):S1-S24. doi: 10.1177/0194599813517083. PMID: 24491310

Recent clinical studies

Etiology

Harjama L, Karvonen V, Kettunen K, Elomaa O, Einarsdottir E, Heikkilä H, Kivirikko S, Ellonen P, Saarela J, Ranki A, Kere J, Hannula-Jouppi K
J Eur Acad Dermatol Venereol 2021 Sep;35(9):1874-1880. Epub 2021 May 21 doi: 10.1111/jdv.17314. PMID: 33914963
Tehrani-Bagha AR
Adv Colloid Interface Sci 2019 Jun;268:114-135. Epub 2019 Mar 13 doi: 10.1016/j.cis.2019.03.006. PMID: 31022590
Kosmidis K, Dassios G
J Pharmacokinet Pharmacodyn 2019 Apr;46(2):165-172. Epub 2019 Mar 18 doi: 10.1007/s10928-019-09625-8. PMID: 30880356
Woods JC, Conradi MS
J Magn Reson 2018 Jul;292:90-98. Epub 2018 Apr 26 doi: 10.1016/j.jmr.2018.04.007. PMID: 29705031Free PMC Article
Zhang C, Zhu MY, Zeng GM, Yu ZG, Cui F, Yang ZZ, Shen LQ
Environ Sci Pollut Res Int 2016 Mar;23(5):4370-86. Epub 2016 Jan 14 doi: 10.1007/s11356-016-6076-8. PMID: 26762937

Diagnosis

Harjama L, Karvonen V, Kettunen K, Elomaa O, Einarsdottir E, Heikkilä H, Kivirikko S, Ellonen P, Saarela J, Ranki A, Kere J, Hannula-Jouppi K
J Eur Acad Dermatol Venereol 2021 Sep;35(9):1874-1880. Epub 2021 May 21 doi: 10.1111/jdv.17314. PMID: 33914963
Mukherjee A, Davis HC, Ramesh P, Lu GJ, Shapiro MG
Prog Nucl Magn Reson Spectrosc 2017 Nov;102-103:32-42. Epub 2017 Jun 3 doi: 10.1016/j.pnmrs.2017.05.002. PMID: 29157492Free PMC Article
Dak P, Ebrahimi A, Swaminathan V, Duarte-Guevara C, Bashir R, Alam MA
Biosensors (Basel) 2016 Apr 14;6(2):14. doi: 10.3390/bios6020014. PMID: 27089377Free PMC Article
Hegewald MJ
Clin Rev Allergy Immunol 2009 Dec;37(3):159-66. Epub 2009 Mar 31 doi: 10.1007/s12016-009-8125-2. PMID: 19330553
Marriott C
Symp Soc Exp Biol 1989;43:163-77. PMID: 2701476

Therapy

Estrada E, Gómez-Gardeñes J, Lacasa L
Proc Natl Acad Sci U S A 2023 Aug;120(31):e2305001120. Epub 2023 Jul 25 doi: 10.1073/pnas.2305001120. PMID: 37490534Free PMC Article
Bermejo M, Sanchez-Dengra B, Gonzalez-Alvarez M, Gonzalez-Alvarez I
Expert Opin Drug Deliv 2020 Jun;17(6):791-803. Epub 2020 Apr 20 doi: 10.1080/17425247.2020.1750593. PMID: 32248713
Nesmelova IV, Melnikova DL, Ranjan V, Skirda VD
Prog Mol Biol Transl Sci 2019;166:85-108. Epub 2019 Jun 8 doi: 10.1016/bs.pmbts.2019.05.004. PMID: 31521238
Le Bihan D
Neuroimage 2019 Feb 15;187:56-67. Epub 2017 Dec 22 doi: 10.1016/j.neuroimage.2017.12.062. PMID: 29277647
Lees KR
Br Med Bull 2000;56(2):401-12. doi: 10.1258/0007142001903049. PMID: 11092089

Prognosis

Hillen T, Loy N, Painter KJ, Thiessen R
J Math Biol 2023 Nov 28;88(1):4. doi: 10.1007/s00285-023-02025-0. PMID: 38015257Free PMC Article
Berlinghieri R, Krajbich I, Maccheroni F, Marinacci M, Pirazzini M
Sci Adv 2023 Aug 25;9(34):eadf1665. Epub 2023 Aug 23 doi: 10.1126/sciadv.adf1665. PMID: 37611107Free PMC Article
Tiu VE, Enache I, Panea CA, Tiu C, Popescu BO
Medicina (Kaunas) 2022 Mar 3;58(3) doi: 10.3390/medicina58030377. PMID: 35334554Free PMC Article
Weibel ER
Cell Tissue Res 2017 Mar;367(3):413-426. Epub 2016 Dec 16 doi: 10.1007/s00441-016-2541-4. PMID: 27981379
Nielsen JB, Benfeldt E, Holmgaard R
Curr Probl Dermatol 2016;49:103-11. Epub 2016 Feb 4 doi: 10.1159/000441549. PMID: 26844902

Clinical prediction guides

Hillen T, Loy N, Painter KJ, Thiessen R
J Math Biol 2023 Nov 28;88(1):4. doi: 10.1007/s00285-023-02025-0. PMID: 38015257Free PMC Article
Harjama L, Karvonen V, Kettunen K, Elomaa O, Einarsdottir E, Heikkilä H, Kivirikko S, Ellonen P, Saarela J, Ranki A, Kere J, Hannula-Jouppi K
J Eur Acad Dermatol Venereol 2021 Sep;35(9):1874-1880. Epub 2021 May 21 doi: 10.1111/jdv.17314. PMID: 33914963
Panebianco V, Narumi Y, Altun E, Bochner BH, Efstathiou JA, Hafeez S, Huddart R, Kennish S, Lerner S, Montironi R, Muglia VF, Salomon G, Thomas S, Vargas HA, Witjes JA, Takeuchi M, Barentsz J, Catto JWF
Eur Urol 2018 Sep;74(3):294-306. Epub 2018 May 10 doi: 10.1016/j.eururo.2018.04.029. PMID: 29755006Free PMC Article
Weibel ER
Cell Tissue Res 2017 Mar;367(3):413-426. Epub 2016 Dec 16 doi: 10.1007/s00441-016-2541-4. PMID: 27981379
Hegewald MJ
Clin Rev Allergy Immunol 2009 Dec;37(3):159-66. Epub 2009 Mar 31 doi: 10.1007/s12016-009-8125-2. PMID: 19330553

Recent systematic reviews

Chen Y, Wang Y, Song Z, Fan Y, Gao T, Tang X
Ageing Res Rev 2023 Jun;87:101911. Epub 2023 Mar 15 doi: 10.1016/j.arr.2023.101911. PMID: 36931328
Torres-Castro R, Vasconcello-Castillo L, Alsina-Restoy X, Solis-Navarro L, Burgos F, Puppo H, Vilaró J
Pulmonology 2021 Jul-Aug;27(4):328-337. Epub 2020 Nov 25 doi: 10.1016/j.pulmoe.2020.10.013. PMID: 33262076Free PMC Article
Patanè FG, Liberto A, Maria Maglitto AN, Malandrino P, Esposito M, Amico F, Cocimano G, Rosi GL, Condorelli D, Nunno ND, Montana A
Medicina (Kaunas) 2020 Nov 11;56(11) doi: 10.3390/medicina56110606. PMID: 33187340Free PMC Article
Sandroni C, D'Arrigo S, Cacciola S, Hoedemaekers CWE, Kamps MJA, Oddo M, Taccone FS, Di Rocco A, Meijer FJA, Westhall E, Antonelli M, Soar J, Nolan JP, Cronberg T
Intensive Care Med 2020 Oct;46(10):1803-1851. Epub 2020 Sep 11 doi: 10.1007/s00134-020-06198-w. PMID: 32915254Free PMC Article
Sarmiento A, Diaz-Castro J, Pulido-Moran M, Kajarabille N, Guisado R, Ochoa JJ
Curr Drug Metab 2016;17(4):345-58. doi: 10.2174/1389200216666151103115654. PMID: 26526835

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...