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Chapter 92: Hydroa Vacciniforme

Bonnie W. Leung; Travis W. Vandergriff

HYDROA VACCINIFORME

Therapeutic Approach

Hydroa vacciniforme (HV) is a rare, chronic, scarring photodermatosis with onset typically occurring in childhood associated with Epstein-Barr virus infection. It is characterized by photoinduced papules and vesicles with invariably pox-like scarring after healing. Controlled clinical trials for HV therapy have not been done, and avoidance of ultraviolet radiation is the only established therapy. Evidence for most other therapies remains anecdotal. See Table 92-1.

Table 92-1Hydroa Vacciniforme Treatment Table
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Table 92-1 Hydroa Vacciniforme Treatment Table
MEDICATION NAME INDICATION MECHANISM OF ACTION DOSING ADVERSE EFFECTS RECOMMENDED MONITORING LEVEL OF EVIDENCE (REFERENCE)
Topical Therapy
Broad-spectrum sunscreen and sun avoidance   Sun protection Regular application daily Skin irritation None III1
Systemic Therapy
Acyclovir/valacyclovir Patients with chronic EBV infection Anti-viral 28-72 mg/kg/24 h, divided doses2 Headache, GI upset, renal dosing None III2
Dietary fish oil   Anti-inflammatory 4-5 g/d, 3 mo None None III3,4
Procedural Therapy
Narrowband UVB   Induction of tolerance and immunosuppression Daily ×10 d or 3 times weekly for 5 wk1,5 May cause disease exacerbation None III5,6
Psoralen and UVA In older patients Induction of tolerance and immunosuppression Optimal dosing schedule not established May cause disease exacerbation None III1,7,8

Levels of evidence are based on the Journal of the American Academy of Dermatology guidelines: level IA evidence includes evidence from meta-analysis of randomized controlled trials; level IB evidence includes evidence from ≥1 randomized controlled trial; level IIA evidence includes evidence from ≥1 controlled study without randomization; level IIB evidence includes evidence from ≥1 other type of experimental study; level III evidence includes evidence from nonexperimental descriptive studies, such as comparative studies, correlation studies, and case control studies; and level IV evidence includes evidence from expert committee reports or opinions or clinical experience of respected authorities, or both.

REFERENCES

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1. +
Sonnex  TS, Hawk  JL. Hydroa vacciniforme: a review of ten cases. Br J Dermatol. 1988;118(1):101–108. doi:10.1111/j.1365-2133.1988.tb01757.x 
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2. +
Lysell  J, Wiegleb Edström  D, Linde  A,  et al. Antiviral therapy in children with hydroa vacciniforme. Acta Derm Venereol. 2009;89(4):393–397. doi:10.2340/00015555-0670 
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3. +
Durbec  F, Reguiaï  Z, Léonard  F, Pluot  M, Bernard  P. Efficacy of ω-3 polyunsaturated fatty acids for the treatment of refractory hydroa vacciniforme. Pediatr Dermatol. 2012;29(1):118–119. doi:10.1111/j.1525-1470.2011.01486.x 
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4. +
Rhodes  LE, White  SI. Dietary fish oil as a photoprotective agent in hydroa vacciniforme. Br J Dermatol. 1998;138(1):173–178. doi:10.1046/j.1365-2133.1998.02047.x 
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5. +
Gupta  G, Man  I, Kemmett  D. Hydroa vacciniforme: a clinical and follow-up study of 17 cases. J Am Acad Dermatol. 2000;42(2 Pt 1):208–213. doi:10.1016/S0190-9622(00)90127-0 
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6. +
Collins  P, Ferguson  J. Narrow-band UVB (TL-01) phototherapy: an effective preventative treatment for the photodermatoses. Br J Dermatol. 1995;132(6):956–963. doi:10.1111/j.1365-2133.1995.tb16955.x 
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