Indian Journal of Dermatology
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E-IJD® - CORRESPONDENCE
Year : 2022  |  Volume : 67  |  Issue : 4  |  Page : 482
Aquagenic keratoderma with no palmar involvement and no complete resolution after drying: An unusual clinical presentation


1 From the Department of Medicine, Dermatology Unit, Celio Military Hospital; Dermatology Clinic, Department of Clinical Internal, Anesthesiological and Cardiovascular Sciences, Sapienza University of Rome, Rome, Italy
2 Primary Care Pediatrician, Rome, Italy
3 From the Department of Medicine, Dermatology Unit, Celio Military Hospital, Rome, Italy

Date of Web Publication2-Nov-2022

Correspondence Address:
Alessandro Laghi
From the Department of Medicine, Dermatology Unit, Celio Military Hospital; Dermatology Clinic, Department of Clinical Internal, Anesthesiological and Cardiovascular Sciences, Sapienza University of Rome, Rome
Italy
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.ijd_941_20

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How to cite this article:
Laghi A, Masetti A, Nunno DD, Astorino S, Mandese V. Aquagenic keratoderma with no palmar involvement and no complete resolution after drying: An unusual clinical presentation. Indian J Dermatol 2022;67:482

How to cite this URL:
Laghi A, Masetti A, Nunno DD, Astorino S, Mandese V. Aquagenic keratoderma with no palmar involvement and no complete resolution after drying: An unusual clinical presentation. Indian J Dermatol [serial online] 2022 [cited 2022 Dec 8];67:482. Available from: https://www.e-ijd.org/text.asp?2022/67/4/482/360369




Sir,

Aquagenic keratoderma (AK) is a rare and benign skin disorder consisting in a transient water reactive papulotranslucent acrokeratoderma.[1] Although palmar surfaces are commonly involved, infrequently they might be spared.[1] Pruritus, burning sensation and pain are possible. Exposure time and water temperature can affect the rate and intensity of manifestations. Even though pathogenesis of AK is poorly understood, mutations in genes codifying for ions channels, especially in cystic fibrosis gene (CFTR), are sometimes associated.[2] We examined a 9-year-old Caucasian girl, born out of nonconsanguineous marriage, diagnosed with Attention Deficit Hyperactivity Disorder (ADHD). She had been suffering for few weeks from loss of dermatoglyphics 15 seconds after water exposure at 15°C; no treatments nor further examinations had been prescribed. Clinical examination revealed hyper-wrinkling of all the ventral aspects of the distal phalanges (especially on 2nd and 3rd finger) and whitish-erythematous plaques in correspondence with the metacarpophalangeal joints [Figure 1]. Even if both hands were entirely immersed in the water, palms were not involved. Itching was localized on all these lesions and spared the intact skin. Hyperhidrosis on the palms was not noted. 30 min after the drying, eroded erythematous plaques persisted, but the whitish halo disappeared. No actual therapies and no familiar history of the same manifestations or of cystic fibrosis were reported.
Figure 1: Aquagenic keratoderma of the right hand. All the right hand was immersed in the water. The photo was not easy to take since the patient's comorbidity (ADHD)

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Considering the suggestive history and the comorbidity, we decided not to run a skin biopsy, or a genetic test, and a clinical diagnosis of AK was made. Pediatric keratoderma caused or worsened by water is generally genetically determined and involve mainly palmar surfaces.

For instance, hereditary papulotranslucent acrokeratoderma, an asymptomatic autosomal dominant (AD) condition, consists in persistent palmar papules, worsened by water contact, appearing in the childhood and accompanied by palmar hyperhidrosis.[3] In our case, only erythematous plaques persisted, and no marked hyperhidrosis was noted.

In our patient, the persistence of the erythematous eroded plaques was probably due to her hyperactive attitude because she scratched off the superficial skin of the distal phalanges, making them more reactive to water than palmar surfaces [Figure 2]. This behaviour could justify this unusual AK presentation.
Figure 2: Aquagenic keratoderma. The photo, taken some days later by the parents, proves the migratory presentation of the eroded lesions

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AD inherited mutations in AQP5, a gene codifying for water-channel protein aquaporin-5, cause a palmoplantar keratoderma that appears spongy and white after water exposure.[3] Although this is the closest differential diagnosis, familiar history was negative in our case.

A spongy white reactivity to water is also present in Nagashima-type palmoplantar keratosis, an autosomal recessive palmoplantar keratoderma due to mutations of SERPINB7, a gene normally encoding for a serine protease inhibitor that avoids water permeation through the skin.[3] Nevertheless, our patient was Caucasian, and the keratoderma was transient.

AK is considered a rare and benign skin disorder, but its diagnosis is probably overlooked: the frequency of CFTR mutation carriers varies from 1/28 to 1/84, depending on the population,[4] and AK could appear in 25% of them.[2] Probably AK should be considered as an umbrella term under which we find various presentations, even unilateral,[5] that sometimes are missed by physicians not familiar with this clinical suspect.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Xia Q. Aquagenic acrokeratoderma: Case report with no involvement of the palms. Int J Dermatol. 2012;51:1391-3.  Back to cited text no. 1
    
2.
Gild R, Clay CD, Morey S. Aquagenic wrinkling of the palms in cystic fibrosis and the cystic fibrosis carrier state: A case–control study. Br J Dermatol. 2010;163:1082-4.  Back to cited text no. 2
    
3.
Paller AS, Mancini AJ. Hurwitz Clinical Pediatric Dermatology. 5th ed. Toronto: Elsevier Inc.; 2016.  Back to cited text no. 3
    
4.
Rohlfs EM, Zhou Z, Heim RA, Nagan N, Rosenblum LS, Flynn K, et al. Cystic fibrosis carrier testing in an ethnically diverse US population. Clin Chem. 2011;57:841-8.  Back to cited text no. 4
    
5.
Erkek E. Unilateral transient reactive papulotranslucent acrokeratoderma in a child. Pediatr Dermatol. 2007;24:564–6.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2]



 

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