Frequency of aquagenic palmoplantar keratoderma in cystic fibrosis: a new sign of cystic fibrosis?
N. Garçon-Michel
Department of Dermatology, Brest University Hospital, 29200 Brest, France
Search for more papers by this authorA.-M. Roguedas-Contios
Department of Dermatology, Brest University Hospital, 29200 Brest, France
Search for more papers by this authorG. Rault
Centre of Competence on Cystic Fibrosis of Perharidy, Roscoff, France
Search for more papers by this authorJ. Le Bihan
Centre of Competence on Cystic Fibrosis of Perharidy, Roscoff, France
Search for more papers by this authorS. Ramel
Centre of Competence on Cystic Fibrosis of Perharidy, Roscoff, France
Search for more papers by this authorK. Revert
Centre of Competence on Cystic Fibrosis of Perharidy, Roscoff, France
Search for more papers by this authorA. Dirou
Centre of Competence on Cystic Fibrosis of Perharidy, Roscoff, France
Search for more papers by this authorL. Misery
Department of Dermatology, Brest University Hospital, 29200 Brest, France
Search for more papers by this authorN. Garçon-Michel
Department of Dermatology, Brest University Hospital, 29200 Brest, France
Search for more papers by this authorA.-M. Roguedas-Contios
Department of Dermatology, Brest University Hospital, 29200 Brest, France
Search for more papers by this authorG. Rault
Centre of Competence on Cystic Fibrosis of Perharidy, Roscoff, France
Search for more papers by this authorJ. Le Bihan
Centre of Competence on Cystic Fibrosis of Perharidy, Roscoff, France
Search for more papers by this authorS. Ramel
Centre of Competence on Cystic Fibrosis of Perharidy, Roscoff, France
Search for more papers by this authorK. Revert
Centre of Competence on Cystic Fibrosis of Perharidy, Roscoff, France
Search for more papers by this authorA. Dirou
Centre of Competence on Cystic Fibrosis of Perharidy, Roscoff, France
Search for more papers by this authorL. Misery
Department of Dermatology, Brest University Hospital, 29200 Brest, France
Search for more papers by this authorConflicts of interest None declared.
Summary
Background Aquagenic palmoplantar keratoderma (APPK), also known as aquagenic wrinkling of the palms, is characterized by oedema of palms and/or soles, whitish papules, hyperwrinkling and sometimes pruritus or pain after water immersion. Its frequency in the general population is unknown. About 40 cases have been reported to date, including some among patients with cystic fibrosis (CF) or CF heterozygotes.
Objectives To determine the frequency of APPK among patients with CF.
Methods Twenty-seven patients from the Centre of Competence on Cystic Fibrosis of Roscoff were examined by a dermatologist after immersion of the palms in water for 2–3 min.
Results The frequency of APPK was 41% (11 of 27 patients). Some patients had not previously noticed the lesions. The frequency was higher among inpatients than outpatients. We suspect that occlusion (caused by the gloves worn by inpatients) can explain this difference. The number of patients included in this study is not sufficient to draw any conclusions concerning the type of CF mutation and its impact on the frequency of APPK.
Conclusions APPK is frequent among patients with CF and, thus, should be considered a sign of CF. APPK is underdiagnosed because physicians usually do not look for it. CF screening should be considered for any patient presenting with these symptoms, followed by genetic counselling if necessary.
References
- 1 Yan AC, Aasi SZ, Alms WJ et al. Aquagenic palmoplantar keratoderma. J Am Acad Dermatol 2001; 44: 696–9.
- 2 Betlloch I, Vergara G, Albares MP et al. Aquagenic keratoderma. J Eur Acad Dermatol Venereol 2003; 17: 306–7.
- 3 Diba VC, Cormack GC, Burrows NP. Botulinum toxin is helpful in aquagenic palmoplantar keratoderma. Br J Dermatol 2005; 152: 394–5.
- 4 Saray Y, Deniz S. Familial aquagenic acrokeratoderma: case reports and review of the literature. Int J Dermatol 2005; 44: 906–9.
- 5 Vildósola S, Ugalde A. Queratodermia acuagenica inducida por celecoxib. Actas Dermosifiliogr 2005; 96: 537–9.
- 6 Corgibet F. Kératodermie acrale aquagénique. Nouv Dermatol 2007; 26: 154–5.
- 7 Bardazzi F, Savoia F, Dika E et al. Acquired aquagenic keratoderma. Pediatr Dermatol 2007; 24: 197–8.
- 8 Davis LS, Woody CM. Idiopathic aquagenic wrinkling of the palms. Pediatr Dermatol 2004; 21: 180.
- 9 Katz KA, Yan AC, Turner ML. Aquagenic wrinkling of the palms in patients with cystic fibrosis homozygous for the delta F508 CFTR mutation. Arch Dermatol 2005; 141: 621–4.
- 10 Khuu PT, Duncan KO, Kwan A et al. Unilateral aquagenic wrinkling of the palms associated with aspirin intake. Arch Dermatol 2006; 142: 1661–2.
- 11 Lim KS. Aquagenic wrinkling of the palms in a boy with congenital cardiac anomaly. J Eur Acad Dermatol Venereol 2007; 21: 985–6.
- 12 Gild R, Clay CD. Aquagenic wrinkling of palms in a cystic fibrosis carrier. Australas J Dermatol 2008; 49: 19–20.
- 13 English JC, McCollough ML. Transient reactive papulotranslucent acrokeratoderma. J Am Acad Dermatol 1996; 34: 686–7.
- 14 Lowes MA, Khaira GS, Holt D. Transient reactive papulotranslucent acrokeratoderma associated with cystic fibrosis. Australas J Dermatol 2000; 41: 172–4.
- 15 Erkek E. Unilateral transient reactive papulotranslucent acrokeratoderma in a child. Pediatr Dermatol 2007; 24: 564–6.
- 16 Yalçin B, Artüz F, Toy GG et al. Acquired aquagenic papulotranslucent acrokeratoderma. J Eur Acad Dermatol Venereol 2005; 19: 654–6.
- 17 MacCormack MA, Wiss K, Malhotra R. Aquagenic syringeal acrokeratoderma: report of two teenage cases. J Am Acad Dermatol 2001; 45: 124–6.
- 18 Itin PH, Lautenschlager S. Aquagenic syringeal acrokeratoderma (transient reactive papulotranslucent acrokeratoderma). Dermatology 2002; 204: 8–11.
- 19 Schmults C, Sidhu G, Urbanek W. Aquagenic syringeal acrokeratoderma. Dermatol Online J 2002; 9: 27.
- 20 Conde-Salazar L, Angulo J, Gonzalez-Guerra E et al. Acroqueratodermia siringea acuagenica. Presentacion de dos casos. Actas Dermosifiliogr 2006; 97: 275–7.
- 21 Baldwin BT, Prakash A, Fenske NA, Messina J. Aquagenic syringeal acrokeratoderma: report of a case with histologic findings. J Am Acad Dermatol 2006; 54: 899–902.
- 22 Sais G, Bigata X, Admella C. Aquagenic syringeal acrokeratoderma. Actas Dermosifiliogr 2007; 98: 69–70.
- 23 Kocatürk E, Kavala M, Bûyükbabani N, Türkoglu Z. Whitish papules on the palm. Int J Dermatol 2007; 46: 736–7.
- 24 Neri I, Bianchi F, Patrizi A. Transient aquagenic palmar hyperwrinkling: the first instance reported in a young boy. Pediatr Dermatol 2006; 23: 39–42.
- 25 Seitz CS, Gaigi Z, Brocker EB, Trautmann A. Painful wrinkles in the bathtub: association with hyperhidrosis and cystic fibrosis. Dermatology 2008; 216: 222–6.
- 26 Elliott RB. Wrinkling of skin in cystic fibrosis. Lancet 1974; ii:108 (Letter).
- 27 Johns MK. Skin wrinkling in cystic fibrosis. Med Biol Illus 1975; 25: 205–10.
- 28 Norman AP, Mall ML, Johns MK. Skin wrinkling in cystic fibrosis. Lancet 1974; ii: 358–9.
- 29 Onwukwe MF, Mihm MC Jr, Toda K. Hereditary papulotranslucent acrokeratoderma. A new variant of familial punctate keratoderma? Arch Dermatol 1973; 108: 108–10.
- 30 Carder KR, Weston WL. Rofecoxib-induced instant aquagenic wrinkling of the palms. Pediatr Dermatol 2002; 19: 353–5.
- 31 Moynahan EJ. Skin wrinkling in cystic fibrosis. Lancet 1974; ii:907 (Letter).
- 32 Garçon N, Roguedas AM, Misery L et al. Kératodermie palmo plantaire aquagénique chez un patient hétérozygote pour la mutation DeltaF 508 de la mucoviscidose. Ann Dermatol Venereol 2008; 135: 232–4.
- 33 Corvol H, Brouard J, Clément A. Les gènes modificateurs dans la mucoviscidose. Médecine Thérapeutique Pédiatrie 2005; 8: 150–5.
- 34 Di Sant’Agnese PA, Darling RC, Perera GA et al. Abnormal electrolyte composition of sweat in cystic fibrosis of the pancreas: clinical implications and relationship to the disease. Pediatrics 1953; 12: 549–63.
- 35 Yigit H, Selimoglu MA, Altinkaynak S. Sweat test results in children with primary protein energy malnutrition. J Pediatr Gastroenterol Nutr 2003; 37: 242–5.
- 36 Catella-Lawson F, McAdam B, Morrison BW et al. Effects of specific inhibition of cyclooxygenase-2 on sodium balance, hemodynamics, and vasoactive eicosanoids. J Pharmacol Exp Ther 1999; 289: 735–41.
- 37 Leong J, Hughes-Fulford M, Rakhlin N et al. Cyclooxygenases in human and mouse skin and cultured human keratinocytes: association of COX-2 expression with human keratinocyte differentiation. Exp Cell Res 1996; 224: 79–87.
- 38
Müller-Decker K,
Reinerth G,
Krieg P
et al.
Prostaglandin-H-synthase isozyme expression in normal and neoplastic human skin.
Int J Cancer
1999; 82: 648–56.
10.1002/(SICI)1097-0215(19990827)82:5<648::AID-IJC6>3.0.CO;2-D CAS PubMed Web of Science® Google Scholar
- 39 Spreca A, Simonetti S, Rambotti MG. Atrial natriuretic peptide and guanylin-activated guanylate cyclase isoforms in human sweat glands. Histochem J 2000; 32: 725–31.
- 40 Berk DR, Ciliberto HM, Sweet SC et al. Aquagenic wrinkling of the palms in cystic fibrosis: comparison with controls and genotype–phenotype correlations. Arch Dermatol 2009; 145: 1296–9.
