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Palmoplantar hyperhidrosis(HYPRPP)

MedGen UID:
224808
Concept ID:
C1274743
Disease or Syndrome
Synonyms: HYPERHIDROSIS PALMARIS ET PLANTARIS; HYPERHIDROSIS, PRIMARY PALMAR; HYPRPP
SNOMED CT: Palmoplantar hyperhidrosis (403375001)
 
HPO: HP:0007410
Monarch Initiative: MONDO:0007754
OMIM®: 144110

Definition

Hyperhidrosis palmaris et plantaris (HYPRPP) is characterized by excessive perspiration of the eccrine sweat gland in the palm, sole, and axilla. Perspiration in those affected may be aggravated by emotional stimuli (summary by Higashimoto et al., 2006). Stolman (1998) noted that hyperhidrosis may be complicated by skin maceration as well as secondary microbial infections, and that treatment modalities are associated with complications. [from OMIM]

Clinical features

From HPO
Palmoplantar hyperhidrosis
MedGen UID:
224808
Concept ID:
C1274743
Disease or Syndrome
Hyperhidrosis palmaris et plantaris (HYPRPP) is characterized by excessive perspiration of the eccrine sweat gland in the palm, sole, and axilla. Perspiration in those affected may be aggravated by emotional stimuli (summary by Higashimoto et al., 2006). Stolman (1998) noted that hyperhidrosis may be complicated by skin maceration as well as secondary microbial infections, and that treatment modalities are associated with complications.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPalmoplantar hyperhidrosis

Conditions with this feature

Keratolytic winter erythema
MedGen UID:
98359
Concept ID:
C0406756
Congenital Abnormality
Keratolytic winter erythema, also known as Oudtshoorn skin disease, manifests during childhood with recurrent episodes of palmoplantar erythema and centrifugal epidermal peeling. Lateral and dorsal aspects of the hands and feet can be involved. A less common finding is a slowly migratory, annular erythema that is seen mostly on the extremities. Between flares, the skin may appear unremarkable. Hyperhidrosis, associated with a pungent odor, is invariably present, and itching can occur. Peeling is preceded by the formation of dry blisters due to keratolysis, whereas formation of vesicles or bullae is rare. Cold weather, moisture, febrile diseases, and physical and mental stress can trigger exacerbations. In severely affected individuals, skin manifestations persist unremittingly. Penetrance of the disease is high, but expressivity is variable, even within the same family (summary by Ngcungcu et al., 2017).
Odonto-onycho-dermal dysplasia
MedGen UID:
208666
Concept ID:
C0796093
Disease or Syndrome
Odontoonychodermal dysplasia (OODD) is an autosomal recessive disorder characterized by dry hair, severe hypodontia, smooth tongue with marked reduction of fungiform and filiform papillae, onychodysplasia, hyperkeratosis of the palms and soles, hypo- and hyperhidrosis of the skin, and atrophic patches on the face (summary by Adaimy et al., 2007; Yu et al., 2019).
Palmoplantar hyperhidrosis
MedGen UID:
224808
Concept ID:
C1274743
Disease or Syndrome
Hyperhidrosis palmaris et plantaris (HYPRPP) is characterized by excessive perspiration of the eccrine sweat gland in the palm, sole, and axilla. Perspiration in those affected may be aggravated by emotional stimuli (summary by Higashimoto et al., 2006). Stolman (1998) noted that hyperhidrosis may be complicated by skin maceration as well as secondary microbial infections, and that treatment modalities are associated with complications.
Pachyonychia congenita 2
MedGen UID:
314107
Concept ID:
C1721007
Disease or Syndrome
Pachyonychia congenita (PC) is characterized by hypertrophic nail dystrophy, painful palmoplantar keratoderma and blistering, oral leukokeratosis, pilosebaceous cysts (including steatocystoma and vellus hair cysts), palmoplantar hyperhydrosis, and follicular keratoses on the trunk and extremities.
Nonsyndromic congenital nail disorder 5
MedGen UID:
318994
Concept ID:
C1833909
Disease or Syndrome
Hereditary distal onycholysis is an autosomal dominant nail disorder characterized by a decreased rate of growth of the nail, scleronychia, and a straight or concave proximal edge of detachment. Clinical features may include palmoplantar hyperhydrosis and marked sensitivity of the fingers to cold (summary by Bazex et al., 1990). Hereditary distal onycholysis is referred to here as nonsyndromic congenital nail disorder-5 (NDNC5). For a list of other nonsyndromic congenital nail disorders and a discussion of genetic heterogeneity, see NDNC1 (161050).
Tooth agenesis, selective, 4
MedGen UID:
372057
Concept ID:
C1835492
Disease or Syndrome
Any tooth agenesis in which the cause of the disease is a mutation in the WNT10A gene.
Peeling skin syndrome 1
MedGen UID:
336530
Concept ID:
C1849193
Disease or Syndrome
A group of rare autosomal recessive forms of ichthyosis with clinical characteristics of superficial, asymptomatic, spontaneous peeling of the skin and histologically by a shedding of the outer layers of the epidermis. Presents with either an acral or a generalised distribution.
Palmoplantar keratoderma-XX sex reversal-predisposition to squamous cell carcinoma syndrome
MedGen UID:
461281
Concept ID:
C3149931
Disease or Syndrome
Palmoplantar keratoderma-XX sex reversal-predisposition to squamous cell carcinoma syndrome is characterised by sex reversal in males with a 46, XX (SRY-negative) karyotype, palmoplantar hyperkeratosis and a predisposition to squamous cell carcinoma. To date, five cases (four of whom were brothers) have been described. The aetiology is unknown.
Palmoplantar keratoderma, Nagashima type
MedGen UID:
816402
Concept ID:
C3810072
Disease or Syndrome
Nagashima-type palmoplantar keratoderma is an autosomal recessive nonsyndromic diffuse palmoplantar keratosis, first described by Nagashima (1977) in the Japanese literature. It is characterized by well-demarcated diffuse erythematous hyperkeratosis that extends onto the dorsal surfaces of the palms and feet and the Achilles tendon area. Involvement of the elbows and knees has also been reported, and there is a high frequency of hyperhidrosis on the palms and soles. In contrast to other types of transgressive diffuse hyperkeratosis such as mal de Meleda (248300), PPKN shows only mild hyperkeratosis that is nonprogressive after the second decade and does not involve flexion contractures or constricting bands (summary by Kubo et al., 2013). For a discussion of phenotypic and genetic heterogeneity of palmoplantar keratoderma, see epidermolytic PPK (144200).
Neurodevelopmental disorder with progressive microcephaly, spasticity, and brain anomalies
MedGen UID:
1380260
Concept ID:
C4479631
Disease or Syndrome
Neurodevelopmental disorder with progressive microcephaly, spasticity, and brain anomalies (NDMSBA) is an autosomal recessive neurodevelopmental disorder characterized by infantile onset of progressive microcephaly and spasticity and severe global developmental delay resulting in profoundly impaired intellectual development and severely impaired or absent motor function. More variable features include seizures and optic atrophy. Brain imaging may show myelinating abnormalities and white matter lesions consistent with a leukoencephalopathy, as well as structural anomalies, including thin corpus callosum, gyral abnormalities, and cerebral or cerebellar atrophy. Some patients die in early childhood (summary by Falik Zaccai et al., 2017 and Hall et al., 2017).

Professional guidelines

PubMed

Campanati A, Gregoriou S, Milia-Argyti A, Kontochristopoulos G, Radi G, Diotallevi F, Martina E, Offidani A
Expert Opin Pharmacother 2022 Jul;23(10):1217-1231. Epub 2022 Jul 10 doi: 10.1080/14656566.2022.2083499. PMID: 35686667
Arora G, Kassir M, Patil A, Sadeghi P, Gold MH, Adatto M, Grabbe S, Goldust M
J Cosmet Dermatol 2022 Jan;21(1):62-70. Epub 2021 Aug 20 doi: 10.1111/jocd.14378. PMID: 34416078
Walling HW, Swick BL
Am J Clin Dermatol 2011 Oct 1;12(5):285-95. doi: 10.2165/11587870-000000000-00000. PMID: 21714579

Recent clinical studies

Etiology

Brandt E, Harjama L, Elomaa O, Saarela J, Donner K, Lappalainen K, Kivirikko S, Ranki A, Kere J, Kettunen K, Hannula-Jouppi K
J Eur Acad Dermatol Venereol 2024 Feb;38(2):413-418. Epub 2023 Sep 22 doi: 10.1111/jdv.19498. PMID: 37684051
Hügül H, Özkoca D, Kutlubay Z
J Cosmet Dermatol 2022 May;21(5):1948-1952. Epub 2022 Feb 25 doi: 10.1111/jocd.14845. PMID: 35156765
Yazbek G, Ishy A, Alexandrino da Silva MF, Sposato Louzada AC, de Campos JRM, Kauffman P, Tedde ML, Puech-Leão P, Pêgo-Fernandes PM, Wolosker N
Ann Vasc Surg 2021 Nov;77:25-30. Epub 2021 Aug 17 doi: 10.1016/j.avsg.2021.05.014. PMID: 34411664
Walling HW, Swick BL
Am J Clin Dermatol 2011 Oct 1;12(5):285-95. doi: 10.2165/11587870-000000000-00000. PMID: 21714579
Tetteh HA, Groth SS, Kast T, Whitson BA, Radosevich DM, Klopp AC, D'Cunha J, Maddaus MA, Andrade RS
Ann Thorac Surg 2009 Jan;87(1):267-74; discussion 274-5. doi: 10.1016/j.athoracsur.2008.10.028. PMID: 19101310

Diagnosis

Hügül H, Özkoca D, Kutlubay Z
J Cosmet Dermatol 2022 May;21(5):1948-1952. Epub 2022 Feb 25 doi: 10.1111/jocd.14845. PMID: 35156765
Arora G, Kassir M, Patil A, Sadeghi P, Gold MH, Adatto M, Grabbe S, Goldust M
J Cosmet Dermatol 2022 Jan;21(1):62-70. Epub 2021 Aug 20 doi: 10.1111/jocd.14378. PMID: 34416078
Yazbek G, Ishy A, Alexandrino da Silva MF, Sposato Louzada AC, de Campos JRM, Kauffman P, Tedde ML, Puech-Leão P, Pêgo-Fernandes PM, Wolosker N
Ann Vasc Surg 2021 Nov;77:25-30. Epub 2021 Aug 17 doi: 10.1016/j.avsg.2021.05.014. PMID: 34411664
Scarff CE
Aust Fam Physician 2009 Sep;38(9):666-9. PMID: 19893792
Thomas I, Brown J, Vafaie J, Schwartz RA
Am Fam Physician 2004 Mar 1;69(5):1117-20. PMID: 15023010

Therapy

Saravanan R, Baalann KP
Pan Afr Med J 2022;41:289. Epub 2022 Apr 8 doi: 10.11604/pamj.2022.41.289.26065. PMID: 35855037Free PMC Article
Campanati A, Gregoriou S, Milia-Argyti A, Kontochristopoulos G, Radi G, Diotallevi F, Martina E, Offidani A
Expert Opin Pharmacother 2022 Jul;23(10):1217-1231. Epub 2022 Jul 10 doi: 10.1080/14656566.2022.2083499. PMID: 35686667
K Panda P, Mehta S, Sharawat IK
Trop Doct 2021 Oct;51(4):624-625. Epub 2021 Apr 15 doi: 10.1177/00494755211007966. PMID: 33853461
Walling HW, Swick BL
Am J Clin Dermatol 2011 Oct 1;12(5):285-95. doi: 10.2165/11587870-000000000-00000. PMID: 21714579
Thomas I, Brown J, Vafaie J, Schwartz RA
Am Fam Physician 2004 Mar 1;69(5):1117-20. PMID: 15023010

Prognosis

Masarwa H, Salama N, Turk A, Abuawad M, Hawari A, Ziyadeh J, Al Zabadi H
Ann Vasc Surg 2024 Nov;108:317-324. Epub 2024 Jul 11 doi: 10.1016/j.avsg.2024.05.013. PMID: 39002894
Yazbek G, Ishy A, Alexandrino da Silva MF, Sposato Louzada AC, de Campos JRM, Kauffman P, Tedde ML, Puech-Leão P, Pêgo-Fernandes PM, Wolosker N
Ann Vasc Surg 2021 Nov;77:25-30. Epub 2021 Aug 17 doi: 10.1016/j.avsg.2021.05.014. PMID: 34411664
Dogruk Kacar S, Ozuguz P, Eroglu S, Polat S, Karaca S
Cutan Ocul Toxicol 2014 Dec;33(4):313-6. Epub 2014 Jan 9 doi: 10.3109/15569527.2013.875559. PMID: 24405389
Reisfeld R, Pasternack GA, Daniels PD, Basseri E, Nishi GK, Berliner KI
Am Surg 2013 Aug;79(8):845-53. PMID: 23896256
Baumgartner FJ, Reyes M, Sarkisyan GG, Iglesias A, Reyes E
Ann Thorac Surg 2011 Dec;92(6):2015-9. doi: 10.1016/j.athoracsur.2011.07.083. PMID: 22115211

Clinical prediction guides

Vural AT, Özcan D, Güleç AT
J Cosmet Dermatol 2022 May;21(5):2162-2168. Epub 2022 Jan 4 doi: 10.1111/jocd.14723. PMID: 34982502
Yazbek G, Ishy A, Alexandrino da Silva MF, Sposato Louzada AC, de Campos JRM, Kauffman P, Tedde ML, Puech-Leão P, Pêgo-Fernandes PM, Wolosker N
Ann Vasc Surg 2021 Nov;77:25-30. Epub 2021 Aug 17 doi: 10.1016/j.avsg.2021.05.014. PMID: 34411664
Kim HM, Lee MJ, Lee MH, Lee H
Skin Res Technol 2020 Jul;26(4):577-583. Epub 2020 Jan 10 doi: 10.1111/srt.12835. PMID: 31922304
Dogruk Kacar S, Ozuguz P, Eroglu S, Polat S, Karaca S
Cutan Ocul Toxicol 2014 Dec;33(4):313-6. Epub 2014 Jan 9 doi: 10.3109/15569527.2013.875559. PMID: 24405389
Tetteh HA, Groth SS, Kast T, Whitson BA, Radosevich DM, Klopp AC, D'Cunha J, Maddaus MA, Andrade RS
Ann Thorac Surg 2009 Jan;87(1):267-74; discussion 274-5. doi: 10.1016/j.athoracsur.2008.10.028. PMID: 19101310

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