Transient Reactive Papulotranslucent Acrokeratoderma: A Report of 3 Cases Showing Excellent Response to Topical Calcipotriene
Transient reactive papulotransucent acrokeratoderma (TRPA) is a rare disorder characterized by edema and wrinkling of the palms following 5 to 10 minutes of contact with water that typically resolves within 1 hour after cessation of exposure. Based on encouraging preliminary observations in 3 patients, the authors recommend therapeutic trials of topical calcipotriene in the treatment of TRPA.
To the Editor:
Transient reactive papulotranslucent acrokeratoderma (TRPA) is a rare disorder that also has been described using the terms aquagenic syringeal acrokeratoderma, aquagenic palmoplantar keratoderma, aquagenic acrokeratoderma, aquagenic papulotranslucent acrokeratoderma, and aquagenic wrinkling of the palms.1 It was initially described in 1996 by English and McCollough,2 and since then fewer than 100 cases have been reported.1-12
A 38-year-old man presented with prominent palmar hyperhidrosis with whitish papules on the palms of 10 days’ duration. The lesions were exacerbated following exposure to water but were asymptomatic aside from their unsightly cosmetic appearance. Dermatologic examination revealed translucent, whitish, pebbly papules confined to the central palmar creases (Figure 1) that were intensified following a 5-minute water immersion test.
Histopathologic examination of a punch biopsy specimen from the right palm revealed orthokeratotic hyperkeratosis and slight hypergranulosis in the epidermis (Figure 2). Subtle eccrine glandular hyperplasia was evident in the dermis (Figure 3). Periodic acid–Schiff staining was negative. Based on the clinical findings and results of the water immersion test, a diagnosis of TRPA was made. A therapeutic trial of calcipotriene ointment 0.005% twice daily was initiated and resulted in dramatic clearance of the lesions within 2 weeks (Figure 4). At 1-month follow-up, the patient was virtually free of all symptoms and no disease recurrence was noted at 5-year follow-up.
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Figure 1. Whitish, pebbly papules confined to the central palmar creases in a 38-year-old man. | Figure 2. Orthokeratotic hyperkeratosis and mild hypergranulosis was noted in the epidermis (H&E, original magnification ×100). | ||
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| Figure 3. Luminal dilatation in the eccrine glands with a prominence of glandular epithelial cells, which displayed abundant cytoplasm with a granular appearance (H&E, original magnification ×100). | Figure 4. Remarkable response to calcipotriene ointment 0.005%. The white punctuate scar indicates the previous punch biopsy site. |
A 25-year-old woman presented with whitish plaques on the palms of 7 days’ duration. She reported frequent use of household cleansers in the month prior to presentation. The lesions were associated with prominent hyperhidrosis, pruritus, and a tingling sensation in the palms. Dermatologic examination revealed confluent, macerated, white, pavement stone–like papules with prominent puncta around the palmar flexures on both palms. Lesions were exacerbated after a 5-minute water immersion test (Figure 5).
The patient refused skin biopsy, and conservative treatment with a barrier cream and limited water exposure were of no benefit. Based on the clinical findings and results of the water immersion test, a diagnosis of TRPA was made. Due to the excellent outcome experienced in treating the previous patient, a trial of calcipotriene ointment 0.005% twice daily was initiated, and the patient reported complete resolution of signs and symptoms within the initial 2 weeks of treatment. Treatment was terminated at 1-month follow-up.
A 6-year-old boy presented with swollen, itchy palms of 2 months’ duration that the patient described as “wet” and “white.” Due to a recent epidemic of bird flu, the patient’s mother had advised him to use liquid cleansers and antiseptic gels on the hands for the past 2 months, which is when the symptoms on the palms started to develop. On dermatologic examination, whitish, cobblestonelike papules were noted near the palmar creases in association with profuse hyperhidrosis (Figure 6). Based on the clinical findings, a diagnosis of TRPA was made. Biopsy was not attempted and the patient was treated with calcipotriene ointment 0.005% twice daily. At 1-month follow-up, complete clearance of the lesions was noted.
Transient reactive papulotranslucent acrokeratoderma is an acquired and sporadic disorder that can occur in both sexes.2,4,6,8-11 Onset generally occurs during adolescence or young adulthood.1,3,8,9 Clinically, TRPA is characterized by edema and wrinkling of the palms following 5 to 10 minutes of contact with water that typically resolves within 1 hour after cessation of exposure.2,3,6-8,10 The “hand-in-the-bucket” sign refers to accentuation of physical findings upon immersion of the hand in water.6,10,11 Patients frequently report itching, burning, or tingling sensations in the affected areas.2,4,6,7,9,11 Transient reactive papulotranslucent acrokeratoderma usually affects the palms in a diffuse, bilateral, and symmetrical pattern,2,4,6-10 but cases showing involvement of the soles,6,7 marginal distribution of lesions,3 unilateral involvement,1 and prominence on the dorsal fingers5 also have been reported. The natural disease course involves reactive episodes and quiescent intervals.2,7,9 Spontaneous resolution of TRPA has been reported.4,6,8
The histological characteristics described in previous reports involve compact orthohyperkeratosis with dilated acrosyringia,2-6,9,11 hyperkeratosis and hypergranulosis in the epidermis,4,8,12 and eccrine glandular hyperplasia.5,12 Alternatively, the skin may appear completely normal on histology.1,7