Aquagenic keratoderma.

BACKGROUND: Aquagenic keratoderma is triggered in the palms and soles after contact with water, and is characterized by the appearance of translucent papules forming macerated plaques. It may be associated with medications and diseases such as cystic fibrosis, atopy, and malnutrition, or be idiopathic. CASE REPORT: We describe the case of a 17-year-old female patient with chronic functional abdominal pain. She presented with a 2-month history of "wrinkling" of palms after contact with water. After stimulation with water, palmar hyperlinearity and whitish, translucent papules forming macerated-looking plaques with a central depression were observed. Dermoscopically, we observed whitish and anfractive structures with coral appearance and microdroplets of water. In the histological study, we observed continuous hyperkeratosis and acrosyringium dilation from the middle dermis to the stratum corneum. With the clinical presentation and histological findings, aquagenic keratoderma was diagnosed, and treatment was started with partial improvement. CONCLUSIONS: Aquagenic keratoderma is an underdiagnosed entity. Despite its indolent course, it could be considered as a marker of a systemic disease such as cystic fibrosis. Since the discussion about the terminology of the disease has arisen, we considered adjusting to a descriptive nomenclature, proposing the term whitish macerated aquagenic plaques of the acrosyringium. It is necessary to continue reporting these cases to understand the disease better and offer adequate management and comprehensive follow-up to the patients.

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 «arrugamiento¼ 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 acrosiringio¼. Es necesario continuar reportando estos casos para comprender mejor la enfermedad, ofrecer un manejo adecuado y dar seguimiento integral a los pacientes.

Queratodermia acuagénica / Aquagenic keratoderma

Resumen 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 «arrugamiento¼ 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 acrosiringio¼. Es necesario continuar reportando estos casos para comprender mejor la enfermedad, ofrecer un manejo adecuado y dar seguimiento integral a los pacientes.

Abstract Background: Aquagenic keratoderma is triggered in the palms and soles after contact with water, and is characterized by the appearance of translucent papules forming macerated plaques. It may be associated with medications and diseases such as cystic fibrosis, atopy, and malnutrition, or be idiopathic. Case report: We describe the case of a 17-year-old female patient with chronic functional abdominal pain. She presented with a 2-month history of "wrinkling" of palms after contact with water. After stimulation with water, palmar hyperlinearity and whitish, translucent papules forming macerated-looking plaques with a central depression were observed. Dermoscopically, we observed whitish and anfractive structures with coral appearance and microdroplets of water. In the histological study, we observed continuous hyperkeratosis and acrosyringium dilation from the middle dermis to the stratum corneum. With the clinical presentation and histological findings, aquagenic keratoderma was diagnosed, and treatment was started with partial improvement. Conclusions: Aquagenic keratoderma is an underdiagnosed entity. Despite its indolent course, it could be considered as a marker of a systemic disease such as cystic fibrosis. Since the discussion about the terminology of the disease has arisen, we considered adjusting to a descriptive nomenclature, proposing the term whitish macerated aquagenic plaques of the acrosyringium. It is necessary to continue reporting these cases to understand the disease better and offer adequate management and comprehensive follow-up to the patients.

La queratodermia acuagénica: actualización terapéutica / Aquagenic Keratoderma: Treatment Update

La queratodermia acuagénica (QA) es una afectación dermatológica adquirida poco frecuente que se caracteriza por la aparición de edema y pápulas blanquecinas-translúcidas desencadenado por la inmersión o contacto con el agua. Se han descrito casos asociados a fármacos, hiperhidrosis y a fibrosis quística. Los objetivos del estudio son evaluar la efectividad de los distintos tratamientos existentes para la QA. Realizamos una revisión de la literatura existente al respecto hasta el momento, incluyendo series de casos y reportes de caso. El tratamiento de la QA es efectivo en las formas asociadas a hiperhidrosis. La iontoforesis del agua del grifo, la simpatectomía torácica endoscópica, las inyecciones de toxina botulínica y la oxibutinina son efectivas en las formas refractarias. La aplicación tópica de ácido salicílico o sales de aluminio es efectiva, pero resulta poco eficaz como tratamiento de mantenimiento. Probablemente la mejor alternativa para el tratamiento de la QA sea la oxibutinina 5mg/día vo. Se ha observado que los efectos fisiopatológicos de los antiinflamatorios no esteroideos en la QA podrían justificar el uso de las prostaglandinas como un tratamiento dirigido de la enfermedad. Se necesitan estudios adicionales para fortalecer estas deducciones y abordar las incertidumbres restantes (AU)

Aquagenic keratoderma is an uncommon acquired dermatosis characterized by edema and whitish-translucent papules triggered by immersion or contact with water. Cases have been described in association with certain medications, hyperhidrosis, and cystic fibrosis. The aim of this review is to evaluate the effectiveness of different treatments for aquagenic keratoderma. We reviewed the literature and analyzed treatments for aquagenic keratoderma described in case series and reports. Aquagenic keratoderma associated with hyperhidrosis can be treated effectively. Tap water iontophoresis, endoscopic thoracic sympathectomy, botulinum toxin injections, and oxybutynin are effective against refractory forms. Topical salicylic acid and aluminum salts are effective, but of little value as maintenance therapy. Oral oxybutynin 5 mg/d is probably the best option for treating aquagenic keratoderma. The reported pathophysiological effects of nonsteroidal anti inflammatory drugs in this setting suggest that the use of prostaglandins might be justified. Additional studies are needed to investigate these hypotheses and resolve other questions (AU)

[Translated article] Aquagenic Keratoderma: Treatment Update / La queratodermia acuagénica: actualización terapéutica

Aquagenic keratoderma is an uncommon acquired dermatosis characterized by edema and whitish-translucent papules triggered by immersion or contact with water. Cases have been described in association with certain medications, hyperhidrosis, and cystic fibrosis. The aim of this review is to evaluate the effectiveness of different treatments for aquagenic keratoderma. We reviewed the literature and analyzed treatments for aquagenic keratoderma described in case series and reports. Aquagenic keratoderma associated with hyperhidrosis can be treated effectively. Tap water iontophoresis, endoscopic thoracic sympathectomy, botulinum toxin injections, and oxybutynin are effective against refractory forms. Topical salicylic acid and aluminum salts are effective, but of little value as maintenance therapy. Oral oxybutynin 5 mg/d is probably the best option for treating aquagenic keratoderma. The reported pathophysiological effects of nonsteroidal anti inflammatory drugs in this setting suggest that the use of prostaglandins might be justified. Additional studies are needed to investigate these hypotheses and resolve other questions (AU)

La queratodermia acuagénica (QA) es una afectación dermatológica adquirida poco frecuente que se caracteriza por la aparición de edema y pápulas blanquecinas-translúcidas desencadenado por la inmersión o contacto con el agua. Se han descrito casos asociados a fármacos, hiperhidrosis y a fibrosis quística. Los objetivos del estudio son evaluar la efectividad de los distintos tratamientos existentes para la QA. Realizamos una revisión de la literatura existente al respecto hasta el momento, incluyendo series de casos y reportes de caso. El tratamiento de la QA es efectivo en las formas asociadas a hiperhidrosis. La iontoforesis del agua del grifo, la simpatectomía torácica endoscópica, las inyecciones de toxina botulínica y la oxibutinina son efectivas en las formas refractarias. La aplicación tópica de ácido salicílico o sales de aluminio es efectiva, pero resulta poco eficaz como tratamiento de mantenimiento. Probablemente la mejor alternativa para el tratamiento de la QA sea la oxibutinina 5mg/día vo. Se ha observado que los efectos fisiopatológicos de los antiinflamatorios no esteroideos en la QA podrían justificar el uso de las prostaglandinas como un tratamiento dirigido de la enfermedad. Se necesitan estudios adicionales para fortalecer estas deducciones y abordar las incertidumbres restantes (AU)

Aquagenic Keratoderma: Treatment Update. / La queratodermia acuagénica: actualización terapéutica.

Aquagenic keratoderma is an uncommon acquired dermatosis characterized by edema and whitish-translucent papules triggered by immersion or contact with water. Cases have been described in association with certain medications, hyperhidrosis, and cystic fibrosis. The aim of this review is to evaluate the effectiveness of different treatments for aquagenic keratoderma. We reviewed the literature and analyzed treatments for aquagenic keratoderma described in case series and reports. Aquagenic keratoderma associated with hyperhidrosis can be treated effectively. Tap water iontophoresis, endoscopic thoracic sympathectomy, botulinum toxin injections, and oxybutynin are effective against refractory forms. Topical salicylic acid and aluminum salts are effective, but of little value as maintenance therapy. Oral oxybutynin 5 mg/d is probably the best option for treating aquagenic keratoderma. The reported pathophysiological effects of nonsteroidal anti inflammatory drugs in this setting suggest that the use of prostaglandins might be justified. Additional studies are needed to investigate these hypotheses and resolve other questions.

Therapeutic Use of Botulinum Neurotoxins in Dermatology: Systematic Review.

Botulinum toxin is a superfamily of neurotoxins produced by the bacterium Clostridium Botulinum with well-established efficacy and safety profile in focal idiopathic hyperhidrosis. Recently, botulinum toxins have also been used in many other skin diseases, in off label regimen. The objective of this manuscript is to review and analyze the main therapeutic applications of botulinum toxins in skin diseases. A systematic review of the published data was conducted, following Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. Botulinum toxins present several label and off-label indications of interest for dermatologists. The best-reported evidence concerns focal idiopathic hyperhidrosis, Raynaud phenomenon, suppurative hidradenitis, Hailey-Hailey disease, epidermolysis bullosa simplex Weber-Cockayne type, Darier's disease, pachyonychia congenita, aquagenic keratoderma, alopecia, psoriasis, notalgia paresthetica, facial erythema and flushing, and oily skin. Further clinical trials are still needed to better understand the real efficacy and safety of these applications and to standardize injection and doses protocols for off label applications.

[Clinical and evolutionary characteristics of a child with aquagenic keratoderma: A retrospective study of 12 patients]. / Caractéristiques cliniques et évolutives de la kératodermie aquagénique de l'enfant : étude rétrospective de 12 cas.

INTRODUCTION: Aquagenic keratoderma (AK) is a rare condition characterized by wrinkled and edematous appearance of the skin of the hands occurring within minutes of immersion in water. Other than in a setting of cystic fibrosis, AK has rarely been reported in children, with only 13 clinical cases on record. Many clinicians are unfamiliar with AK and have fears relating to the association with cystic fibrosis The aim of this study is to describe the characteristics and to discuss management of the disease. METHODS: Retrospective, multicentre study, including children aged under 16 years presenting AK. RESULTS: 12 children were included. KA started at a mean age of 9.25 years (range: 20 months to 15 years). Clinical appearance and mode of onset were classical, with the palms being more severely affected than the soles. Pruritus or pain were reported in six cases. The median impact on daily life was 1.5/10. Some of the children underwent investigations: two had a negative sweat test, three had molecular analysis of the gene CFTR: one was negative and two had a heterozygote mutation. The course of the disease was variable: eight stabilizations, two exacerbations, one cure and one improvement. DISCUSSION: This is the first series on childhood KA. Clinical characteristics were similar to those seen in adults. Impact was moderate and the disease course was variable. Systematic medical check-up for cystic fibrosis does not appear warranted in children since to date, cystic fibrosis has not been diagnosed in any patients presenting AK alone. CONCLUSION: AK is rare in children and should not cause erroneous concern, and improvement can occur.