Aquagenic Wrinkling of Skin: A Screening Test for Cystic Fibrosis

Singh, Anant Kumar; Lodha, Rakesh; Shastri, Shivaram; Sethuraman, Gomathy; Sreedevi, K N; Kabra, Madhulika; Kabra, S. K.

doi:10.1007/s13312-019-1481-y

Cited by 11 publications

(19 citation statements)

References 14 publications

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“…Finally, the CF-het group might have had a slightly higher time to manifest aquagenic wrinkling because this trait could be altered as age increases. 25 In conclusion, our study detected a strong association between AWP and CF. BIW test could be performed very easily and can be used as an initial screening tool for individuals with symptoms and signs compatible with CF.…”

Section: Discussionsupporting

confidence: 51%

“…The utility of AWP as a screening test was studied by Singh et al 25 In their study, time to develop aquagenic wrinkling was checked in 54 children referred for sweat chloride test. A total of 20 children had a sweat chloride value of ≥60 mEq/L.…”

Section: Discussionmentioning

confidence: 99%

“…Secondly, CF mutation analysis was not performed for the control group, therefore we might have included in it a few carriers despite the strict exclusion criteria. Finally, the CF‐het group might have had a slightly higher time to manifest aquagenic wrinkling because this trait could be altered as age increases 25 …”

Section: Discussionmentioning

confidence: 99%

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Aquagenic wrinkling of the palms after brief immersion to water test as a screening tool for cystic fibrosis diagnosis

Alexopoulos

Chouliaras

Kakourou

et al. 2021

Acad Dermatol Venereol

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Background Aquagenic wrinkling of the palms (AWP) is an excessive and early palmar wrinkling occurring after Brief Immersion to Water (BIW), and has been reported as a frequent finding among cystic fibrosis (CF) patients.Objectives To evaluate and assess the diagnostic performance of BIW test as an initial screening tool for CF diagnosis. Methods We measured AWP in CF patients, CF-heterozygotes (CF-het) and normal controls. The AWP parameters of palmar wrinkling, oedema, papules, pruritus and pain were assessed at 3, 7 and 11 min after a BIW test was performed for all the participants. Statistical analyses explored the progression of AWP in time for the three groups and assessed the diagnostic performance of BIW test as a diagnostic screening tool for CF.Results A total of 250 individuals (100 CF patients, their 50 CF-het parents, 100 healthy controls) were included in the analysis. The average age in years (mean AE SD) was 10.4 AE 4.0 for CF, 35.9 AE 6.1 for CF-het and 10.5 AE 4.0 for controls. The rate of positives for AWP at 3 min among CF patients, CF-het and controls was 68%, 8% and 0%, respectively (P < 0.01). Kaplan-Meier analysis showed a clear trend towards earlier appearance of all five parameters in the direction controls < hetCF < CF (P values <0.01). The best diagnostic performance in detecting between CF patients and non-CF was achieved by the presence of papules and wrinkling at 7 min (sensitivity/specificity: 94.0%/98.3% and 100.0%/ 92.0%, respectively).Conclusions A strong association between AWP and CF was detected. AWP after BIW could be elicited easily and possibly can be used as an initial screening tool to assess if an individual with symptoms and signs that raise the likelihood of CF is a CF patient.

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Section: Discussionsupporting

confidence: 51%

Section: Discussionmentioning

confidence: 99%

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Aquagenic wrinkling of the palms after brief immersion to water test as a screening tool for cystic fibrosis diagnosis

Alexopoulos

Chouliaras

Kakourou

et al. 2021

Acad Dermatol Venereol

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“…En el artículo solamente se presenta una imagen clínica, lo que impide sumar su estadística a la reportada en el resto de la literatura internacional, además de no haber otro estudio que replique los hallazgos 2,[20][21][22][23] . En otro estudio, Singh et al 24 concluyeron que los niños con fibrosis quística desarrollan arrugamiento acuagénico más rápido que los controles sanos, por lo que este signo puede considerarse como marcador de fibrosis quística. Con base en estas observaciones, se podría afirmar que la asociación entre el arrugamiento acuagénico y la fibrosis quística es factible, a diferencia de la queratodermia acuagénica, que hasta el momento ha resultado controversial.…”

Section: B Aunclassified

Queratodermia acuagénica

Marín-Hernández¹,

García-Alonso²,

Cruz-Flores³

et al. 2022

BMHIM

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Introducción: La queratodermia acuagénica se desencadena tras el contacto de las palmas de las manos y las plantas de los pies con el agua. Se caracteriza por la aparición de pápulas translúcidas que forman placas de aspecto macerado. Puede asociarse con el consumo de ciertos medicamentos y con afecciones como la fibrosis quística, la atopia y la desnutrición, o ser idiopática. Caso clínico: Se describe el caso de una paciente de 17 años con dolor abdominal crónico funcional. Presentó una dermatosis de 2 meses de evolución que afectaba las palmas con «arrugamiento» después del contacto con el agua. Tras el estímulo con el agua, se observaron hiperlinealidad palmar y pápulas blanquecinas y translúcidas que formaban placas de aspecto macerado con una depresión central. Dermatoscópicamente se observaron estructuras blanquecinas anfractuosas de apariencia coraliforme y microgotas de agua. En el estudio histológico se observaron hiperqueratosis continua y dilatación del acrosiringio desde la dermis media hasta el estrato córneo. Con el cuadro clínico y los hallazgos histológicos, se confirmó el diagnóstico de queratodermia acuagénica y se inició el tratamiento, con el que se observó una mejoría parcial. Conclusiones: La queratodermia acuagénica es una afección subdiagnosticada y poco reportada. A pesar de cursar de forma indolente, puede considerarse como un marcador de enfermedad sistémica como la fibrosis quística. Ya que existe discusión sobre la nomenclatura de la enfermedad, consideramos ajustarnos a una nomenclatura descriptiva, como «placas blanquecinas y maceradas acuagénicas del acrosiringio». Es necesario continuar reportando estos casos para comprender mejor la enfermedad, ofrecer un manejo adecuado y dar seguimiento integral a los pacientes.

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“…Various typical clinical features like steatorrhea, rectal prolapse, metabolic alkalosis, dyselectrolytemia, and failure to thrive in children with recurrent or persistent pneumonia may point toward a diagnosis of CF 3 . Aquagenic wrinkling (wrinkling of hands‐on dipping in water for 3 min) has been reported to have a sensitivity of 81% and specificity of 57% for the diagnosis of CF 7 . Combination of these parameters has not been studied for the diagnosis of CF.…”

Section: Introductionmentioning

confidence: 99%

Development of algorithm for diagnosis of cystic fibrosis in absence of sweat chloride testing in resource‐limited setting

Sahoo

Dhochak

Jat

et al. 2022

Pediatric Pulmonology

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Objective: To develop a diagnostic algorithm for cystic fibrosis (CF) in the setting of unavailability of sweat chloride, based on clinical features and basic laboratory investigations. Methods:In a prospective observational study, we enrolled children with recurrent/persistent pneumonia with either malabsorption or poor growth, undergoing a sweat chloride test, between January 2019 and December 2020.They were simultaneously evaluated for aquagenic wrinkling of hands, stool fat globules, sputum for bacterial culture, blood gas, and serum electrolytes.Sensitivity and specificity were calculated for parameters having a significant difference between CF and non-CF groups. Scoring systems and algorithms for the diagnosis of CF were developed.Results: Of 134 children enrolled, 46 (34%) had CF. The sensitivity and specificity of various parameters to diagnose CF was: sibling death due to respiratory illness (30.43%, 96.59%), aquagenic wrinkling (76.74%, 47.67%), metabolic alkalosis (17.78%, 94.12%), hyponatremia (28.89%, 89.41%), stool fat globules (38.46%, 81.18%), and presence of Pseudomonas in sputum culture (23.68%, 98.80%). Using coefficients of significant parameters on stepwise logistic regression, the composite score for diagnosis of CF was calculated as: 3X sibling death due to respiratory illness + 1.5X hyponatremia + 1.5X metabolic alkalosis + 1.5X aquagenic wrinkling + 1X stool fat globules + 2.5X presence of Pseudomonas in sputum culture (each of the variables scores 0 or 1 for absence and presence, respectively). The cut-off of ≥2.5 had sensitivity and specificity of 81.82% and 76.83%, respectively. Conclusions:In resource-limited settings, the proposed diagnostic algorithm can be used for the diagnosis of presumptive CF with fair sensitivity and specificity.

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Aquagenic Wrinkling of Skin: A Screening Test for Cystic Fibrosis

Cited by 11 publications

References 14 publications

Aquagenic wrinkling of the palms after brief immersion to water test as a screening tool for cystic fibrosis diagnosis

Aquagenic wrinkling of the palms after brief immersion to water test as a screening tool for cystic fibrosis diagnosis

Queratodermia acuagénica

Development of algorithm for diagnosis of cystic fibrosis in absence of sweat chloride testing in resource‐limited setting

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