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Vol. 100. Issue 3.
Pages 222-223 (01 March 2024)
Vol. 100. Issue 3.
Pages 222-223 (01 March 2024)
Images in Paediatrics
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Can I touch water if I have cystic fibrosis?
Tengo fibrosis quística: ¿no puedo tocar el agua?
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Ricardo Suárez Camachoa,
Corresponding author
, Nathalie Carreira Sandeb, Nazareth Martinón Torresb, Carlos García Magánc
a Departamento de Pediatría, Hospital Clínico Universitario de Santiago de Compostela, Santiago de Compostela, Spain
b Unidad de Gastroenterología, Hepatología y Nutrición Pediátrica, Hospital Clínico Universitario de Santiago de Compostela, Santiago de Compostela, Spain
c Unidad de Alergia y Neumología Pediátrica, Hospital Clínico Universitario de Santiago de Compostela, Santiago de Compostela, Spain
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A girl aged 4 years with cystic fibrosis (CF, heterozygous F508del/G542X genotype) was brought in for a checkup and it was reported that she developed papular whitish lesions in palms and soles upon contact with water associated with hyperwrinkling (Figs. 1 and 2) and associated pain if she stayed immersed in water for a long time. The symptoms disappeared after drying.

Figures 1 and 2.

Whitish lesions in the palms of both hands and hyperwrinkling developed during bathing.

The patient was referred to the dermatology department, where she received a diagnosis of aquagenic keratoderma1,2 (AK) and prescribed treatment with emollient creams,2,3 which did not alleviate the symptoms.

At age 6 years, she started treatment of CF with elexacaftor-tezacaftor-ivacaftor. After 3 months of treatment, the family reported complete resolution of AK (Fig. 3).

Figure 3.

Resolution of lesions after treatment with elexacaftor-tezacaftor-ivacaftor.

(0.04MB).

Aquagenic keratosis is a little-known cutaneous disease, but its association with CF has been described extensively,2,3 and it has been hypothesised that sweat hypertonicity could lead to to a higher ratio of flux from eccrine glands in the palms.2 It is a clinical diagnosis (whitish papules with a central depression that appear after contact with water, possibly in association with pain1), and the treatment is symptomatic. Although it is a benign disease, it can have an impact on the patient’s quality of life. Therefore, the use of novel CF conductance regulator modulators could be the answer for the treatment of AK.

References
[1]
M.A. Pastor, L. González, L. Kilmurray, P. Bautista, A. López, A.M. Puig.
Queratodermia acuagénica : tres nuevos casos y revisión de la literatura.
Actas Dermosifiliogr, 99 (2008), pp. 399-406
[2]
M. Carbonell Pradas, R. Grimalt Santacana.
Aquagenic keratoderma: Treatment update.
Actas Dermosifiliogr [Internet], 113 (2022), pp. 254-260
[3]
S. Laliena Aznar, M.C. Vera Sáez-Benito, M.T. Cenarro Guerrero, C. Martín de Vicente.
No puedo jugar con agua.
Rev Pediatr Aten Primaria, 21 (2019), pp. 261-263
Copyright © 2024. Asociación Española de Pediatría
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