Rare Oncology News

Disease Profile

Aquagenic syringeal acrokeratoderma

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

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ICD-10

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Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Aquagenic Palmoplantar Keratoderma; Aquagenic wrinkling of the hands; Transient reactive papulotranslucent acrokeratoderma;

Summary

Aquagenic syringeal acrokeratoderma is a rare condition affecting the palms of the hands. It is characterized by the appearance or worsening of a palmar eruption, following brief exposure to water. The palmar eruption is made up of small, white or shining pimples that can conjoin into plaques. The feet are unaffected. Symptoms include a burning pain and a tightening sensation in the palms, as well as too much sweating. There are two variants. Most commonly, it is a temporary and recurrent condition that appears after submersion in water, known as the “hand in the bucket sign,” that gets better within minutes to hours of drying. A less common variant is characterized by persistent lesions that are worsened after water submersion.[1][2][3] The cause of aquagenic syringeal acrokeratoderma is unknown, but likely relates to sweating. Several studies have found that it is present in about 40% to 84% of cystic fibrosis patients and also in carriers, which suggest that it may be caused by mutations in the CFTR gene.[3] It is more often found in young women. Besides cystic fibrosis, it is also seen in wasting (marasmus) and nephrotic syndrome and also with the use of aspirin and other drugs such as rofecoxib and celecoxib.[4] In most cases it does not need any treatment and resolves spontaneously. When necessary, it can be treated with topical aluminum chloride or salicylic acid ointment or with tap water iontophoresis.[3][5]

Learn more

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • DermNet New Zealand is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.

References

  1. Kent JB, Statuta SM, Greer KE & MacKnight JM. Watersport hands. Sports Health. July, 2014; 6(4):360-2.. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4065561/.
  2. Aquagenic wrinkling of the palms. DermNet NZ. 2015; https://www.dermnetnz.org/reactions/wrinkling.html.
  3. Cabrol C & cols. Aquagenic Palmoplantar Keratoderma as a CFTR-related Disorder. Acta Derm Venereol. March 30, 2016; https://www.ncbi.nlm.nih.gov/pubmed/27026144.
  4. Uyar B. Aquagenic Syringeal Acrokeratoderma. Indian J Dermatol. November, 2014; 59(6):632.
  5. Errichetti E & Piccirillo A. Aquagenic Keratoderma Treated with Tap Water Iontophoresis. Indian J Dermatol. March-April, 2015; 60(2):212.