Diagnosis: Aquagenic syringeal acrokeratoderma

The patient was diagnosed with aquagenic syringeal acrokeratoderma, a rare entity in which brief exposure to water results in the formation of pale or translucent edematous papules on the palmar surface of the hands.


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English and McCollough first reported the condition in 1996 as a “transient reactive papulotranslucent acrokeratoderma” in two sisters.1 Although similarities with hereditary papulotranslucent acrokeratoderma were noted, there were distinguishing characteristics. In 2001, MacCormack et al2 coined the term aquagenic syringeal acrokeratoderma to better identify the affected areas and incorporate the peculiar association with water exposure and distinct histopathology (described below). Although the palms are most frequently affected, rare cases involving the soles of the feet, heel,3 and dorsal aspect of the fingers4 have been reported. Onset is usually during the second or third decade of life, and women are more frequently affected than men. Fewer than 20 cases have been reported.5

In most patients with aquagenic syringeal acrokeratoderma, the palmar surface appears normal at baseline. For others, a mild hyperkeratosis may be clinically evident even in the dry state. The palms become pale, edematous, and wrinkled-appearing after soaking in water for several minutes; closer inspection reveals hypopigmented or translucent papules surrounding dilated eccrine ostia coalescing into plaques.

Associated symptoms are variable and include burning, tenderness, itchiness or tightness of the palmar skin, and pain on flexion of the fingers. Histopathologic examination of a punch biopsy of lesional skin reveals orthokeratotic hyperkeratosis with dilation of the eccrine ducts transversing through the dermis and epidermis; some case reports describe degeneration and proliferation of eccrine epithelial cells.4,6 The boggy appearance generally resolves, returning to baseline within minutes to an hour of drying. Because of the transient nature of the condition, patients often present to the office with their hand submerged in water to demonstrate their symptoms; the “hand-in-bucket” sign is characteristic enough to be considered pathognomonic.6 Warm water may be more effective in eliciting the reaction.7

Aquagenic syringeal acrokeratoderma must be distinguished from hereditary papulotranslucent acrokeratoderma. Both conditions begin at or after puberty; involve acral skin; and demonstrate a peculiar wrinkled, edematous reaction to water or sweat exposure. Although the skin findings may be similar, there are distinct differences between these two entities.

Lesions of aquagenic syringeal acrokeratoderma are transient compared with hereditary papulotranslucent acrokeratoderma lesions, which are persistent. Most cases of aquagenic syringeal acrokeratoderma affect the central palm whereas hereditary papulotranslucent acrokeratoderma more often affects the marginal areas and pressure points of the palms or soles. Eccrine ducts are dilated in aquagenic syringeal acrokeratoderma and normal in hereditary papulotranslucent acrokeratoderma. While hereditary papulotranslucent acrokeratoderma is inherited as an autosomal dominant condition, the genetics of aquagenic syringeal acrokeratoderma are uncertain (although English and McCollough suggested that it could be an autosomal recessive trait).1 Hereditary papulotranslucent acrokeratoderma is associated with finely textured hair and an atopic diathesis, whereas aquagenic syringeal acrokeratoderma is not.8

Other conditions to be considered in the differential include aquagenic pruritus and aquadynia. Although such symptoms as burning pain or itchiness are triggered by water exposure in these conditions, there are typically no visible lesions. Patients with aquagenic urticaria develop widespread hives after water exposure, not a transient palmar keratoderma.

Various topical treatments have been reported, including petroleum-based barrier agents to prevent water absorption and an assortment of agents to ameliorate hyperkeratosis or its symptoms. Aluminum chloride has been an effective desiccant for some patients, but inconsistent efficacy5 and accentuation of the burning sensation6 has limited its use. Several keratolytics have had variable success, including urea creams and salicylic acid in petrolatum. Topical retinoids may be a better choice: tazarotene cream 0.1% resulted in near total resolution of hyperkeratosis in one case; although tretinoin gel 0.1% may not be as effective, it may be a more affordable alternative.5 Reports exist suggesting a role for botulinum toxin injections.

Our patient was not bothered by her condition. She was content to use OTC hand creams on an as-needed basis.

Ms. Winslow is a third-year medical student at Virginia Commonwealth University School of Medicine in Richmond. Dr. Nunley is professor of dermatology at Medical College of Virginia Hospitals, also in Richmond. Neither author has any relationship to disclose relating to the content of this article.


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References

  1. English JC 3rd, McCollough ML. Transient reactive papulotranslucent acrokeratoderma. J Am Acad Dermatol. 1996;34:686-687.
  2. MacCormack MA, Wiss K, Malhotra R. Aquagenic syringeal acrokeratoderma: report of two teenage cases. J Am Acad Dermatol. 2001;45:124-126.
  3. Flann S, Pembroke A. A localized form of aquagenic syringeal acrokeratoderma. Clin Exp Dermatol. 2010;35:e147-148.
  4. Yoon TY, Kim KR, Lee JY, Kim MK. Aquagenic syringeal acrokeratoderma: unusual prominence on the dorsal aspect of fingers? Br J Dermatol. 2008;159:486-488.
  5. Shuler M, Zlotoff B, Bang R. Aquagenic syringeal acrokeratoderma responsive to topical retinoid. J Am Acad Dermatol. 2009;60:AB44.
  6. Baldwin BT, Prakash A, Fenske NA, Messina JL. Aquagenic syringeal acrokeratoderma: report of a case with histologic findings. J Am Acad Dermatol. 2006;54:899-902.
  7. Luo DQ, Li Y, Huang YB, et al. Aquagenic syringeal acrokeratoderma in an adult man: case report and review of the literature. Clin Exp Dermatol. 2009;34:e907-909.
  8. Yan AC, Aasi SZ, Alms WJ, et al. Aquagenic palmoplantar keratoderma. J Am Acad Dermatol. 2001;44:696-699.