eMedicine Specialties > Dermatology > Photo-Related Diseases

Hydroa Vacciniforme

Author: Quynh L Sebastian, MD, Clinical Instructor, Division of Dermatology, UCLA David Geffen School of Medicine
Coauthor(s): Raul Del Rosario, MD, Consulting Staff, Surgical Pathology and Dermatopathology, South Coast Medical
Contributor Information and Disclosures

Updated: Jul 24, 2008

Introduction

Background

Hydroa vacciniforme (HV) is a rare, chronic photodermatosis of unknown origin occurring in childhood. Recurrent vesicles on sun-exposed skin that heal with vacciniform or varioliform scarring characterize HV. The histopathologic features are distinctive and demonstrate intraepidermal reticular degeneration and cellular necrosis. Most cases remit spontaneously by late adolescence.

Pathophysiology

The etiology of HV is not known. HV may be a distinct entity distinguished by scarring or may occur within the spectrum of polymorphous light eruption. Skin lesions occur on sun-exposed skin, such as the face, ears, and hands, and they may be accompanied by a mild keratoconjunctivitis, photophobia, or constitutional symptoms.

Frequency

United States

The frequency of HV in the United States is unknown.

International

The frequency of HV varies according to country. In Scotland, for example, the reported prevalence is 0.34 cases per 100,000 people.

Mortality/Morbidity

No mortality is associated with typical HV.

Sex

Females have a higher incidence of HV than males and report earlier onset. Males who are affected have a longer course of disease than females.

Age

HV predominately affects children aged 3-15 years. Cases of HV in infants and elderly persons have also been described.

Clinical

History

  • Mild burning, itching or stinging in exposed sites beginning 30 minutes to 2 hours after sun exposure (common)
    • Vesicles heal with varioliform scarring.
    • The initial onset of lesions occurs in spring with recurrences in summer months.
  • Constitutional symptoms (uncommon)
  • Photophobia (seldom)

Physical

Skin and mucous membranes1 are the primary sites affected by HV.

  • Skin
    • Tense, edematous papules progress to clear, then, cloudy discrete vesicles.
    • Lesions become umbilicated, necrotic papules on an erythematous base.
    • Papules heal with hypopigmented depressed scars.
  • Eye2
    • Mild keratoconjunctivitis
    • Corneal clouding and stellate keratotic precipitates in the cornea, indicating an inflammatory keratitis (one report3 )
  • Other symptoms
    • Photo-onycholysis
    • Limited partial absorption of bone and cartilage in severe HV
    • Earlobe mutilation and flexion contracture of a digit (reported in 1 patient)4

Causes

The development of lesions and their distribution suggest a causal relationship between HV and ultraviolet (UV) exposure, although the pathogenetic mechanism remains unknown.5 Ultraviolet A (UV-A) radiation is most often implicated. Two reports of HV in siblings have been documented, suggesting a genetic component to HV.6 Some cases have occurred in the setting of hematopoietic malignancy.

More on Hydroa Vacciniforme

Overview: Hydroa Vacciniforme
Differential Diagnoses & Workup: Hydroa Vacciniforme
Treatment & Medication: Hydroa Vacciniforme
Follow-up: Hydroa Vacciniforme
Multimedia: Hydroa Vacciniforme
References
[ CLOSE WINDOW ]

References

  1. Yesudian PD, Sharpe GR. Hydroa vacciniforme with oral mucosal involvement. Pediatr Dermatol. Sep-Oct 2004;21(5):555-7. [Medline].

  2. Jeng BH, Margolis TP, Chandra NS, McCalmont TH. Ocular findings as a presenting sign of hydroa vacciniforme. Br J Ophthalmol. Nov 2004;88(11):1478-9. [Medline].

  3. Wisuthsarewong W, Leenutaphong V, Viravan S. Hydroa vacciniforme with ocular involvement. J Med Assoc Thai. Oct 1998;81(10):807-11. [Medline].

  4. Kim WS, Yeo UC, Chun HS, Lee ES. A case of hydroa vacciniforme with unusual ear mutilation. Clin Exp Dermatol. Mar 1998;23(2):70-2. [Medline].

  5. Halasz CL, Leach EE, Walther RR, Poh-Fitzpatrick MB. Hydroa vacciniforme: induction of lesions with ultraviolet A. J Am Acad Dermatol. Feb 1983;8(2):171-6. [Medline].

  6. Annamalai R. Hydroa vacciniforme in three alternate siblings. Arch Dermatol. Feb 1971;103(2):224-5. [Medline].

  7. Iwatsuki K, Ohtsuka M, Akiba H, Kaneko F. Atypical hydroa vacciniforme in childhood: from a smoldering stage to Epstein-Barr virus-associated lymphoid malignancy. J Am Acad Dermatol. Feb 1999;40(2 Pt 1):283-4. [Medline].

  8. Iwatsuki K, Ohtsuka M, Harada H, Han G, Kaneko F. Clinicopathologic manifestations of Epstein-Barr virus-associated cutaneous lymphoproliferative disorders. Arch Dermatol. Sep 1997;133(9):1081-6. [Medline].

  9. Magaña M, Sangüeza P, Gil-Beristain J, Sánchez-Sosa S, Salgado A, Ramón G, et al. Angiocentric cutaneous T-cell lymphoma of childhood (hydroa-like lymphoma): a distinctive type of cutaneous T-cell lymphoma. J Am Acad Dermatol. Apr 1998;38(4):574-9. [Medline].

  10. Tokura Y, Ishihara S, Ohshima K, Hidano A, Koide M, Seo N, et al. Severe mosquito bite hypersensitivity, natural killer cell leukaemia, latent or chronic active Epstein-Barr virus infection and hydroa vacciniforme-like eruption. Br J Dermatol. May 1998;138(5):905-6. [Medline].

  11. Iwatsuki K, Satoh M, Yamamoto T, Oono T, Morizane S, Ohtsuka M, et al. Pathogenic link between hydroa vacciniforme and Epstein-Barr virus-associated hematologic disorders. Arch Dermatol. May 2006;142(5):587-95. [Medline].

  12. Yamamoto T, Tsuji K, Suzuki D, Morizane S, Iwatsuki K. A novel, noninvasive diagnostic probe for hydroa vacciniforme and related disorders: detection of latency-associated Epstein-Barr virus transcripts in the crusts. J Microbiol Methods. Feb 2007;68(2):403-7. [Medline].

  13. Collins P, Ferguson J. Narrow-band UVB (TL-01) phototherapy: an effective preventative treatment for the photodermatoses. Br J Dermatol. Jun 1995;132(6):956-63. [Medline].

  14. Ziering CL, Rabinowitz LG, Esterly NB. Antimalarials for children: indications, toxicities, and guidelines. J Am Acad Dermatol. May 1993;28(5 Pt 1):764-70. [Medline].

  15. Bruderer P, Shahabpour M, Christoffersen S, André J, Ledoux M. Hydroa vacciniforme treated by a combination of beta-carotene and canthaxanthin. Dermatology. 1995;190(4):343-5. [Medline].

  16. Omenn GS, Goodman GE, Thornquist MD, Balmes J, Cullen MR, Glass A, et al. Effects of a combination of beta carotene and vitamin A on lung cancer and cardiovascular disease. N Engl J Med. May 2 1996;334(18):1150-5. [Medline].

  17. The Alpha-Tocopherol, Beta Carotene Cancer Prevention Study Group. The effect of vitamin E and beta carotene on the incidence of lung cancer and other cancers in male smokers. N Engl J Med. Apr 14 1994;330(15):1029-35. [Medline].

  18. Blackwell V, McGregor JM, Hawk JL. Hydroa vacciniforme presenting in an adult successfully treated with cyclosporin A. Clin Exp Dermatol. Mar 1998;23(2):73-6. [Medline].

  19. Rhodes LE, White SI. Dietary fish oil as a photoprotective agent in hydroa vacciniforme. Br J Dermatol. Jan 1998;138(1):173-8. [Medline].

  20. Rhodes LE, Durham BH, Fraser WD, Friedmann PS. Dietary fish oil reduces basal and ultraviolet B-generated PGE2 levels in skin and increases the threshold to provocation of polymorphic light eruption. J Invest Dermatol. Oct 1995;105(4):532-5. [Medline].

  21. Hashizume H, Tokura Y, Oku T, Iwamoto Y, Takigawa M. Photodynamic DNA-breaking activity of serum from patients with various photosensitivity dermatoses. Arch Dermatol Res. 1995;287(6):586-90. [Medline].

  22. Ashurst PJ. Hydroa vacciniforme occurring in association with Hartnup disease. Br J Dermatol. Jul 1969;81(7):486-92. [Medline].

  23. Bickers DR, Demar LK, DeLeo V, Poh-Fitzpatrick MB, Aronberg JM, Harber LC. Hydroa vacciniforme. Arch Dermatol. Aug 1978;114(8):1193-6. [Medline].

  24. De Pietro U, Simoni R, Barbieri C, Girolomoni G. Hydroa vacciniforme persistent in a 60-year-old man. Eur J Dermatol. Jun 1999;9(4):311-2. [Medline].

  25. Ferguson J, Ibbotson S. The idiopathic photodermatoses. Semin Cutan Med Surg. Dec 1999;18(4):257-73. [Medline].

  26. Goldgeier MH, Nordlund JJ, Lucky AW, Sibrack LA, McCarthy MJ, McGuire J. Hydroa vacciniforme: diagnosis and therapy. Arch Dermatol. Aug 1982;118(8):588-91. [Medline].

  27. Gu H, Chang B, Qian H, Li G. A clinical study on severe hydroa vacciniforme. Chin Med J (Engl). Aug 1996;109(8):645-7. [Medline].

  28. Gupta G, Man I, Kemmett D. Hydroa vacciniforme: A clinical and follow-up study of 17 cases. J Am Acad Dermatol. Feb 2000;42(2 Pt 1):208-13. [Medline].

  29. Raza N, Bari AU, Farooq M. Hydroa vacciniforme in an adolescent girl. J Coll Physicians Surg Pak. Jun 2007;17(6):359-61. [Medline].

  30. Ruiz-Maldonado R, Parrilla FM, Orozco-Covarrubias ML, Ridaura C, Tamayo Sanchez L, Duran McKinster C. Edematous, scarring vasculitic panniculitis: a new multisystemic disease with malignant potential. J Am Acad Dermatol. Jan 1995;32(1):37-44. [Medline].

  31. Sato K, Taguchi H, Maeda T, Minami H, Asada Y, Watanabe Y, et al. The primary cytotoxicity in ultraviolet-a-irradiated riboflavin solution is derived from hydrogen peroxide. J Invest Dermatol. Oct 1995;105(4):608-12. [Medline].

  32. Taylor CR, Duke D. Blistering, scarring, and photosensitivity in a male teenager. Arch Dermatol. Oct 1999;135(10):1267, 1270. [Medline].

  33. Yoon TY, Kim YG, Kim JW, Kim MK. Nodal marginal zone lymphoma in association with hydroa vacciniforme-like papulovesicular eruption, hypersensitivity to mosquito bites and insect bite-like reaction. Br J Dermatol. Jul 2005;153(1):210-2. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

Bazin hydroa vacciniforme, HV, photodermatosis

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Quynh L Sebastian, MD, Clinical Instructor, Division of Dermatology, UCLA David Geffen School of Medicine
Quynh L Sebastian, MD is a member of the following medical societies: American Academy of Dermatology, American Society for Dermatologic Surgery, and American Society for Laser Medicine and Surgery
Disclosure: Nothing to disclose.

Coauthor(s)

Raul Del Rosario, MD, Consulting Staff, Surgical Pathology and Dermatopathology, South Coast Medical
Raul Del Rosario, MD is a member of the following medical societies: American Society for Clinical Pathologists
Disclosure: Nothing to disclose.

Medical Editor

Maureen B Poh-Fitzpatrick, MD, Professor Emerita of Dermatology and Special Lecturer, Columbia University; Professor of Medicine (Dermatology), University of Tennessee
Maureen B Poh-Fitzpatrick, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and New York Academy of Medicine
Disclosure: Nothing to disclose.

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Van Perry, MD, Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas Health Science Center
Van Perry, MD is a member of the following medical societies: American Academy of Dermatology and American Society for Laser Medicine and Surgery
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.