Table of Contents
What Is Aquagenic Keratoderma?
Aquagenic keratoderma (AK) is an uncommon skin disorder, sometimes referred to as aquagenic wrinkling of the palms, acquired aquagenic palmoplantar keratoderma, transient reactive papulotranslucent acrokeratoderma, and aquagenic syringeal acrokeratoderma. Its primary symptoms are wrinkles, papules, burning, and soreness following contact with water. There may also be edema of the palms and soles.
The majority of patients affected are female. It is an exaggeration of the typical wrinkling that happens to fingers after they are submerged in water for an extended amount of time. The length of time spent in the water and its temperature affect the rate and severity of lesion development.
Patients with cystic fibrosis or those who carry the cystic fibrosis gene are most likely to experience this condition. Despite not being life-threatening, the illness can have a major adverse effect on a person's quality of life because of discomfort and concerns about appearance.
What Are the Causes of Aquagenic Keratoderma?
There is no recognized cause for the aquagenic wrinkling of the palms. However, several theories exist to shed light on why this occurrence happens:
- Abnormal Functioning of Sweat Gland: One of the condition's contributing factors is the abnormal function of the sweat glands. When exposed to water, the numerous cutaneous sweat glands on the palms and soles may overproduce, resulting in localized swelling and distinctive wrinkles.
- Salt Imbalance in the Skin Cells: It might be brought on by an imbalance in the salt content of the skin cells, which leads to a rise in both transepidermal water loss and water retention in the cells themselves.
- Genetic Predisposition: Certain research indicates a possible hereditary susceptibility, particularly considering the increased occurrence in those with cystic fibrosis or who carry the CFTR (cystic fibrosis transmembrane conductance regulator) gene mutation. Mutations in the CFTR gene, which controls the flow of salt and water into and out of cells, can result in abnormal skin hydration and barrier function.
- Defect in Barrier Function: An impairment in the function of the epidermal barrier that results in heightened water permeability is also considered. The outermost layer of the skin, the stratum corneum, may enlarge quickly as a result, resulting in the evident changes. The osmotic water shift across the stratum corneum may also rise due to the elevated concentration of sweat salts.
- Certain Medications: Aspirin, non-steroidal anti-inflammatory drugs, and some antibiotics have been linked to certain cases of aquagenic keratoderma. This could be because the drugs have imbalanced skin aquaporin, a protein that facilitates the passage of water across cell membranes.
- Frequent Handwashing: There have been recent reports of cases during the COVID-19 (coronavirus disease 2019) epidemic that may have been brought on by frequent hand washing and sanitizing.
What Does Aquagenic Keratoderma Look Like?
The hallmark feature of aquagenic keratoderma is excessive wrinkling and the appearance of oedematous transparent, yellow, or white plaques and papules on the palms following exposure to water. Normal pathological wrinkling of the palmar skin occurs after 11 minutes of hand in the water; however, aquagenic keratoderma typically manifests three minutes in the water and may be accompanied by itching, soreness, tightness, and discomfort. However, salt water does not cause aquagenic keratoderma on feet or hands.
The hand-in-bucket, a classic indicator of acquired aquagenic palmoplantar keratoderma, is the rapid development of these skin lesions following exposure to water. The most typical presentation involves symmetrical involvement of the palms, with the soles occasionally involved as well. The fingers' forehead, heel, and dorsal are additional unusual locations for aquagenic palmoplantar keratoderma, and unilateral involvement is also possible. Most instances get better about 20 minutes after the water exposure is stopped.
Is Aquagenic Keratoderma Dangerous?
In general, aquagenic keratoderma is not regarded as a dangerous condition. Many patients find that the symptoms are not bothersome and the illness is self-limiting. Although it can be painful and result in burning or itching feelings, it is mostly benign and does not pose a significant risk to one's health. Nonetheless, there is a high correlation between it and cystic fibrosis. Therefore, a diagnosis of aquagenic wrinkles should initiate an early cystic fibrosis workup.
How Is Aquagenic Keratoderma Diagnosed?
Aquagenic wrinkling of the palms is typically diagnosed clinically. The water immersion test, which includes submerging the patient's hands in water for a predetermined amount of time (generally three to five minutes) and watching for the development of distinctive skin changes (also known as the hand in the bucket sign), may typically be used to demonstrate the palmar eruption.
A skin punch biopsy might yield corroborating data to validate the diagnosis. Histological observations may show dilated sweat ducts, hyperkeratosis (thickening of the stratum corneum), acanthosis (darkening and thickening of the skin), and occasionally spongiosis (intercellular edema). In certain cases where cystic fibrosis is suspected, genetic testing for CFTR mutation may be carried out.
How Is Aquagenic Keratoderma treated?
The main goals of managing aquagenic keratoderma are symptomatic alleviation and treating any underlying medical issues.
Options for treatment consist of:
- Barrier creams, gloves, and petroleum jelly are general precautions that can be taken to avoid direct exposure to water, but they are mostly unsuccessful.
- Applying 20 percent aluminum chloride hexahydrate once or twice a day could help lessen the severity of aquagenic keratoderma.
- Botulinum toxin injections have reportedly proven beneficial in treating a tiny percentage of instances.
- Some lifestyle adjustments, like limiting extended exposure to water, can aid with symptom management. Additionally, using gloves when engaging in aquatic activities can function as a physical barrier and delay the start of symptoms.
- The severity of this condition can be decreased by managing underlying conditions such as cystic fibrosis and hyperhidrosis with the right drugs and therapies.
Conclusion
Aquagenic keratoderma is a rare skin condition characterized by temporary skin changes that occur when exposed to water. Although not fatal, it can have a profound effect on the lives of the individuals impacted. Despite its close correlation with cystic fibrosis, an aquagenic keratoderma diagnosis should initiate comprehensive cystic fibrosis investigations. Enhanced awareness, precise diagnosis, and efficacious interventions are crucial in overcoming the obstacles presented by this illness.
