Aquagenic syringeal acrokeratoderma
Published Web Locationhttps://doi.org/10.5070/D32p95h8cq
Aquagenic syringeal acrokeratoderma
From the Ronald O. Perelman Department of Dermatology, New York University
Chrysalyne Schmults MD, Gurdip Sidhu MD, and R Walter Urbanek MD
Dermatology Online Journal 9(4): 27
A case of a 32-year-old woman with aquagenic syringeal acrokeratoderma is presented. This case is the eleventh to report this condition. As with previously reported cases, the condition presents in young women and results in edema of the palms with visibly prominent eccrine ducts after brief exposure to water. The patient responded to aluminum chloride applied topically. Prior cases are reviewed.
History.—A 32-year-old woman presented with a 3-month history of a palmar dermatosis. The noted wrinkling and edema of the skin on the palms after soaking in water for 5-10 minutes. The wrinkling and edema are accompanied by sensations of tightness, tingling, pruritus, and mild pain with flexion. The condition begins to resolve 15-20 minutes after exposure to water has ceased. Past medical history is not contributory. The patient takes no medications. No other family members are affected. The condition has improved with application of aluminum chloride solution (Drysol®) nightly under occlusion.
Physical examination.—The initial examination of the palmar surfaces is normal. However, after soaking the hands in water for 10-15 minutes, the volar surface of the hands appears edematous with thick, undulating ridges and visibly dilated eccrine ducts. The arches of the feet have ridges and edema slightly greater than normal after 30 minutes of exposure to water.
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Histopathology.—Hematoxylin-and-eosin stained sections show a slightly thickened, compact orthokeratotic cornified layer within which are dilated acrosyringia. There is slight keratolysis of the cornified layer overlying the acrosyringia and slight dilatation of the eccrine ostia. The remaining epidermis and dermis are unremarkable.
Diagnosis.—Aquagenic syringeal acrokeratoderma.
This case is the eleventh to report aquagenic palmoplantar keratoderma or transient reactive papulotranslucent acrokeratoderma. The first cases are described in 1996 by English and McCollough . They report two sisters with a similar condition, in which translucent, white, confluent papules with dilated puncta became evident on the palms after 3-5 minutes exposure to water. One sister has a slight hyperkeratosis at baseline. Pathologic examination shows dilated eccrine ostia. Subsequently, four reports describe eight additional patients with a similar condition [2, 3, 4, 5]. All patients are female. The age of onset ranges from 9-33 years with all but two patients presenting in their late teens to early twenties. Most patients have disease confined to their hands although involvement of the soles is reported. The pathology reported by Lowes et al. is similar to those described by English et al. Palmar hyperhidrosis is present in three patients, palmar erythema in one, and a concurrent diagnosis of malignant melanoma, and one has cystic fibrosis. Spontaneous amelioration or disappearance of symptoms occured in three patients. Treatment with aluminum chloride was successful for three patients. Only the two sisters initially reported had affected family members.
The cause is unknown. The finding of dilated eccrine ostia has been reproduced in our patient. Our patient has a good response to aluminum chloride, and it appears to be the treatment of choice at this time for this unusually but potentially underdiagnosed condition.
References1. English JC, et al. Transient reactive papulotranslucent acrokeratoderma. J Am Acad Dermatol 1996;34:686.
2. Lowes MA, et al. Transient reactive papulotranslucent acrokeratoderma associated with cystic fibrosis. Australas J Dermatol 2000;41:172.
3. Yan AC, et al. Aquagenic palmoplantar keratoderma. J Am Acad Dermatol 2001;44:696.
4. MacCormack MA, et al. Aquagenic syringeal acrokeratoderma: Report of two teenage cases. J Am Acad Dermatol 2001;45:124.
5. Itin PH, et al. Aquagenic syringeal acrokeratoderma. Dermatology 2002;204:8.
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