Diamond Jubilee year
ISSN: 0019-5154
Indian Journal of Dermatology • Volume 60 • Issue 2 • March-April 2015 • Pages 109-****
Issue highlights • Male genital lichen sclerosus • Immunomodulators in warts • Serendipity in dermatology • Zinc-responsive acral hyperkeratotic dermatosis • Quality of life and psychological morbidity in vitiligo patients
Indian Journal of
• Clinico-mycological study of dermatophyte toenail onychomycosis
Dermatology
• Skin prick test in patients with chronic allergic skin disorders
Volume 60
• Facial angiofibromas treated with topical sirolimus
Cutaneous B cell lymphomas
• Novel uses of skin biopsy punches • Diffuse cutaneous mastocytosis with bullous lesions and pulmonary involvement • Multiple synchronous verrucous carcinomas of the scalp • Secondary syphilid on healed varicella
IJD
®
www.e-ijd.org
Issue 2
March-April 2015
E-IJD THERAPEUTIC ROUND
Aquagenic Keratoderma Treated with Tap Water Iontophoresis Enzo Errichetti, Angelo Piccirillo
Abstract Aquagenic keratoderma (AK) is a rare acquired skin condition characterized by recurrent and transient white papules and plaques associated with a burning sensation, pain, pruritus and/or hyperhidrosis on the palms and more rarely, soles triggered by sweat or contact with water. Often AK cause significant discomfort, thus requiring an appropriate therapy. Topical aluminum‑based products are the most commonly used medications, but they are not always effective. We report a case of AK unresponsive to topical 20% of aluminum chloride successfully treated with tap water iontophoresis. Key Words: Aquagenic keratoderma, tap water iontophoresis, therapy, treatment
From the Department of Dermatology, San Carlo Hospital, Potenza, Italy
Address for correspondence: Dr. Enzo Errichetti, U.O. Dermatologia, Azienda Ospedaliera San Carlo di Potenza, Via P. Petrone 1, 85100 Potenza, Italy. E‑mail:
[email protected]
What was known? 1. Aquagenic keratoderma (AK) is a rare acquired condition consisting in recurrent and transient white papules and plaques associated with a burning sensation, pain, pruritus and/or hyperhidrosis on the palms and more rarely, soles triggered by sweat or contact with water. 2. The pathogenesis of AK is still unknown, despite multiple hypotheses have been proposed including eccrine gland or nerve dysfunction, hyperhidrosis, defective stratum corneum barrier function, increased sweat salt concentration in the stratum corneum, occlusion of the eccrine duct ostia, or weakness of the eccrine duct wall. 3. Often AK cause significant discomfort, thus requiring an appropriate therapy. Topical aluminum‑based products are the most commonly used medications, but they are not always effective. Other anecdotal therapies include salicylic‑acid based products, formalin 3% in alcohol, antihistamines, botulinum toxin injections and iontophoresis. Regarding this last therapy, there is only one report describing its use in AK, but the results were not specified.
Introduction Aquagenic keratoderma (AK), also known as aquagenic palmoplantar keratoderma, aquagenic syringeal keratoderma, aquagenic wrinkling of the palms and aquagenic acrokeratoderma, is a rare condition presenting with recurrent and transient white papules and plaques on the palms and more rarely, soles triggered by sweat or contact with water. AK is predominant in women with a mean age of onset of 21 years. Its etiology is unknown, although about one‑third of cases are associated with cystic fibrosis (CF) and some instances are induced by intake of various drugs, including aspirin, indomethacin, salazopyrin, rofecoxib and celecoxib.[1‑3] Often AK cause significant discomfort, thus requiring an appropriate therapy.[1] Topical aluminum‑based products are the most commonly used medications, but they are not always effective. Other anecdotal therapies include salicylic‑acid based products, formalin 3% in alcohol, antihistamines, topical corticoids, topical erythromycin, barrier agents, botulinum toxin injections and iontophoresis.[3,4] We report a case of AK unresponsive to topical 20% of Access this article online Quick Response Code: Website: www.e‑ijd.org
DOI: 10.4103/0019-5154.152568
aluminum chloride successfully treated with tap water iontophoresis.
Case Report This was a case report of a 30‑year‑old male patient presented with a 3‑year history of a burning sensation and papular lesions on both palms arising after sweating or immersion in water for a few minutes. The manifestations were transient and resolved within 30‑60 min after drying his hands. No other family members presented similar findings. Patient also reported a history of hyperhidrosis, while denied relevant personal pathological antecedents or history of drug intake. Physical examination revealed a normal palmar skin surface. After immersing the hands in water at 15°C for 5 min, small white pebble‑like papules with prominent eccrine ostia were evident [Figure 1a and b] and the patient reported a burning sensation. The lesions and symptoms normalized within 40 min after drying. The history and clinical observations led to the diagnosis of AK. Considering the possibility of association between AK and CF,[5] we decided to perform a sweat test, which showed a normal amount of chloride; the patient declined further genetic testing for CF. He was treated with topical 20% of aluminum chloride applied once daily, without any significant results on AK and hyperhidrosis after 6 weeks of therapy. Therefore, we decided to suspend it and start a new treatment with tap water iontophoresis; both hands in the pronated
Errichetti and Piccirillo: Aquagenic keratoderma treated with iontophoresis
a
b
a
b
Figure 1: (a) Small white pebble‑like papules with prominent eccrine ostia after immersing the hands in water at 15°C for 5 min; (b) magnification of the papules after immersion
Figure 2: (a) A new immersion test after treatment with tap water iontophoresis for 4 weeks shows a notable clinical improvement; (b) magnification of the prior image
position were immersed in tap water and were exposed to controlled electric current (10‑15 mA) for 15 min on Monday, Wednesday and Friday for 4 weeks. No side‑effects were observed, except for the onset of some transient white papules on the palms associated with a tolerable fleeting burning sensation; both findings decreased gradually over the therapeutic sessions. At the end of treatment schedule, we performed a new immersion test, which showed a notable clinical improvement with remission of the burning sensation [Figure 2a and b]. Furthermore, the patient reported a significant improvement of hyperhidrosis. During the subsequent 3‑month follow‑up period, the patient retained the results achieved.
due to increased sweat salt concentration in the stratum corneum.[1] However, this explicative model is hardly applicable in our case, since the amount of chloride in the sweat was normal.
Discussion
In our case, the patient was found to resistant to topical 20% of aluminum chloride, while he had a significant improvement to tap water iontophoresis.
AK is a rare acquired condition consisting in translucent whitish papules and plaques of the palms and more seldom, of the soles which occur after sweating or immersion in water, especially when warm and disappear after drying within minutes to an hour. Most of the cases are bilateral, although instances of unilateral palmar AK have rarely been described in the literature. Often patients report a burning sensation, pain, pruritus and/or hyperhidrosis associated with skin lesions. The diagnosis of AK is mainly clinical and the “hand‑in‑the‑bucket” sign, when patients submerge their hands in water for a time ranging from 2 to 10 min in order to demonstrate the lesions, is an important diagnostic clue.[4] Histopathology is nonspecific and may show orthokeratotic hyperkeratosis and dilated eccrine ducts.[1,4] The main differential diagnosis is hereditary papulotranslucent acrokeratoderma, which appears during puberty with persistent, asymptomatic, yellow‑white, translucent palmoplantar papules and plaques that are unrelated to water exposure.[6] The pathogenesis of AK is still unknown; despite multiple hypotheses have been proposed including eccrine gland or nerve dysfunction, hyperhidrosis, defective stratum corneum barrier function, occlusion of the eccrine duct ostia, or weakness of the eccrine duct wall.[4,7] Furthermore, based on association some cases of AK with CF and intake of aspirin or cyclo‑oxygenase‑2 inhibitors, some of the authors have suggested that AK could be
Although cases of spontaneous remission have been described,[5] in most patients AK tends to persist and cause a significant physical and psychological discomfort, thus requiring an appropriate therapy. Several treatments have been reported including aluminum‑based or salicylic‑acid based products, formalin 3% in alcohol, antihistamines, botulinum toxin injections and iontophoresis.[4] Regarding this last therapy, there is only one report describing its use in AK, but the results were not specified.[8]
The latter has long been known to inhibit sweat production and therefore it is often used for the treatment of hyperhidrosis. It has been suggested that tap water iontophoresis selectively targets areas with high levels of electrolytes because of enhanced current flow. A local electro‑chemical coagulation of proteins occurs in these areas and subsequently disrupts eccrine gland function. Another theory hypothesizes iontophoresis causes interrupted stimulus‑secretion‑coupling that leads to a functional disturbance of sweat secretion.[9] The exactly mechanisms by which this therapy acts upon AK are unknown. Anyhow, on the basis of the foregoing and whereas improving of AK in our case has occurred together with the reduction of sweating, it is possible to speculate that the effects of iontophoresis on AK are attributable to the reduction of sweat production and/or functional changes of eccrine gland.
Conclusion The clinical improvement observed in this report suggests that a protocol based on tap water iontophoresis, as the one exposed here, followed by post‑treatment follow‑up could be considered as a possible therapeutic option for AK, especially for cases unresponsive to topical drugs. Moreover, the successful treatment of AK with tap water
Errichetti and Piccirillo: Aquagenic keratoderma treated with iontophoresis
iontophoresis, which is known to influence the activity of eccrine sweat glands, further supports a possible involvement of these glands in the pathogenesis of this condition, as assumed by some Authors on the basis of the efficacy of other therapies, which act on their function.[4,10] Anyhow, further studies and reports are needed to confirm these assumptions. What is new? 1. The clinical improvement observed in this report suggests that a protocol based on tap water iontophoresis, as the one exposed here, could be considered as a possible therapeutic option for AK, especially for cases unresponsive to topical drugs. 2. The successful treatment of AK with tap water iontophoresis, which is known to influence the activity of eccrine sweat glands, further supports a possible involvement of these glands in the pathogenesis of this condition.
References 1. Luo DQ, Li Y, Huang YB, Wu LC, He DY. Aquagenic syringeal acrokeratoderma in an adult man: Case report and review of the literature. Clin Exp Dermatol 2009;34:e907‑9. 2. Gündüz O, Ozsaraç KÇ, Ercin ME. Aquagenic palmar wrinkling induced by combined use of salazopyrin and indomethacin. Case Rep Dermatol 2013;5:21‑6. 3. Garçon‑Michel N, Roguedas‑Contios AM, Rault G, Le Bihan J, Ramel S, Revert K, et al. Frequency of aquagenic palmoplantar keratoderma in cystic fibrosis: A new sign of cystic fibrosis?
Br J Dermatol 2010;163:162‑6. 4. Houle MC, Al Dhaybi R, Benohanian A. Unilateral aquagenic keratoderma treated with botulinum toxin A. J Dermatol Case Rep 2010;4:1‑5. 5. Coelho‑Macias V, Fernandes S, Lamarão P, Assis‑Pacheco F, Cardoso J. Aquagenic keratoderma associated with a mutation of the cystic fibrosis gene. Rev Port Pneumol 2013;19:125‑8. 6. Xia Q. Aquagenic acrokeratoderma: Case report with no involvement of the palms. Int J Dermatol 2012;51:1391‑3. 7. Syed Z, Wanner M, Ibrahimi OA. Aquagenic wrinkling of the palms: A case report and literature review. Dermatol Online J 2010;16:7. 8. Lowes MA, Khaira GS, Holt D. Transient reactive papulotranslucent acrokeratoderma associated with cystic fibrosis. Australas J Dermatol 2000;41:172‑4. 9. Siah TW, Hampton PJ. The effectiveness of tap water iontophoresis for palmoplantar hyperhidrosis using a Monday, Wednesday, and Friday treatment regime. Dermatol Online J 2013;19:14. 10. Bagazgoitia L, Pérez‑Carmona L, Salgüero I, Harto A, Jaén P. Letter: Aquagenic keratoderma: Successful treatment with botulinum toxin. Dermatol Surg 2010;36:434‑6. How to cite this article: Errichetti E, Piccirillo A. Aquagenic keratoderma treated with tap water iontophoresis. Indian J Dermatol 2015;60:212. Received: September, 2013. Accepted: October, 2013. Source of support: Nil, Conflict of Interest: Nil.
