What are the other Names for this Condition? (Also known as/Synonyms)

• Aquagenic Keratoderma
• Aquagenic Wrinkling of the Hands
• Transient Reactive Papulotranslucent Acrokeratoderma

What is Aquagenic Syringeal Acrokeratoderma? (Definition/Background Information)

• Aquagenic Syringeal Acrokeratoderma is a rare condition affecting the palms of the hands. It is more often found in young women
• 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 in this condition. 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
• 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
• 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
• 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

(Source: Aquagenic Syringeal Acrokeratoderm; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

Who gets Aquagenic Syringeal Acrokeratoderma? (Age and Sex Distribution)

• Aquagenic Syringeal Acrokeratoderma is a rare disorder. The presentation of symptoms may begin in childhood
• Young women are reported to be predominantly affected by the condition
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Aquagenic Syringeal Acrokeratoderma? (Predisposing Factors)

The risk factors for Aquagenic Syringeal Acrokeratoderma may include the following:

• A family history of the condition
• Cystic fibrosis
• Palmar hyperhidrosis
• Palmar erythema
• Allergic rhinitis
• Bronchial asthma
• Malignant melanoma
• Nephrotic syndrome
• Marasmus
• Use of cyclooxygenase 2 (COX2) inhibitor medications, such as
  • Aspirin
  • Celecoxib
  • Rofecoxib
• Use of certain non-steroidal anti-inflammatory drugs (NSAIDs) and antibiotics

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Aquagenic Syringeal Acrokeratoderma? (Etiology)

• The cause of Aquagenic Syringeal Acrokeratoderma is not known at the present time, but it is believed to be related to sweating
• Mutation(s) in CFTR gene may be involved in development of this condition
• The development of symptoms is believed to be mediated either by salt imbalance in the skin leading to water retention, or due to an abnormal regulation of aquaporin-3 (a transmembrane transport regulator) by drugs

What are the Signs and Symptoms of Aquagenic Syringeal Acrokeratoderma?

The signs and symptoms of Aquagenic Syringeal Acrokeratoderma may vary in severity among affected individuals, and include:

• Wrinkling of skin in palms after submersion in water
  • In case of individuals with cystic fibrosis, wrinkling occurs within 3 minutes of submersion of hands in water
  • In individuals, who are carriers of cystic fibrosis, the required time of submersion for skin wrinkling is about 7 minutes
• Formation of white or shiny pimples or papules in the palms, which may be small in size. However, such papules can coalesce to form larger plaques
• Excessive sweating in palms
• A sensation of skin-tightening in the palms
• Itching, pain, tingling or burning sensations, when plaques develop, or when they are present

How is Aquagenic Syringeal Acrokeratoderma Diagnosed?

Aquagenic Syringeal Acrokeratoderma is diagnosed on the basis of the following information:

• Complete physical examination
• Thorough medical history evaluation
• Assessment of signs and symptoms: The individual’s palms may be examined after submersion in water
• Laboratory tests
• Imaging studies
• Biopsy studies, if necessary

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Aquagenic Syringeal Acrokeratoderma?

The complications of Aquagenic Syringeal Acrokeratoderma may include:

• Itching and discomfort with eruption of plaques
• The wrinkled skin may taking longer than the normal time required (less than an hour), to return to normalcy

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Aquagenic Syringeal Acrokeratoderma Treated?

• In most cases, Aquagenic Syringeal Acrokeratoderma does not need any treatment and resolves spontaneously 
• When necessary, the condition can be treated with topical aluminum chloride or salicylic acid ointment or with tap water iontophoresis

(Source: Aquagenic Syringeal Acrokeratoderm; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

• Immersing or bathing affected hand/s in salt water
• Injections of botulinum toxin
• Medications to address itching

How can Aquagenic Syringeal Acrokeratoderma be Prevented?

• Aquagenic Syringeal Acrokeratoderma occurs as a response to submersion of hands in water, and hence, avoiding submersion for long durations may help prevent or minimize plaque development
• Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic disorders
• Regular medical screening at periodic intervals with tests and physical examinations are recommended

What is the Prognosis of Aquagenic Syringeal Acrokeratoderma? (Outcomes/Resolutions)

The prognosis of Aquagenic Syringeal Acrokeratoderma is generally good, since the condition is transient and resolves on its own or with medication.

Additional and Relevant Useful Information for Aquagenic Syringeal Acrokeratoderma:

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/