Hydroa Vacciniforme

Author: Quynh L Sebastian, MD; Chief Editor: Dirk M Elston, MD more...

Updated: Nov 22, 2011

What would you like to print?

Background
Pathophysiology
Epidemiology
Show All

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.

Characteristic vesicular lesions occur on sun-exposed skin and heal with varioliform scarring.

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.

Proceed to Clinical Presentation

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, Dermatopathology, Mission Hospital at Laguna Beach

Raul Del Rosario, MD is a member of the following medical societies: American Society for Clinical Pathology

Disclosure: Nothing to disclose.

Specialty Editor Board

Maureen B Poh-Fitzpatrick, MD Professor Emerita of Dermatology and Special Lecturer, Columbia University College of Physicians and Surgeons; Professor of Medicine (Dermatology), University of Tennessee Health Science Center College of Medicine

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: Lundbeck, Inc. Honoraria Review panel membership; Clinuvel Pharmaceuticals, Ltd. Honoraria Consulting

Michael J Wells, MD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

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.

Van Perry, MD Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas School of Medicine at San Antonio

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.

Catherine M Quirk, MD Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M 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, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References

Yesudian PD, Sharpe GR. Hydroa vacciniforme with oral mucosal involvement. Pediatr Dermatol. Sep-Oct 2004;21(5):555-7. [Medline].
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].
Wisuthsarewong W, Leenutaphong V, Viravan S. Hydroa vacciniforme with ocular involvement. J Med Assoc Thai. Oct 1998;81(10):807-11. [Medline].
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].
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].
Annamalai R. Hydroa vacciniforme in three alternate siblings. Arch Dermatol. Feb 1971;103(2):224-5. [Medline].
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].
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].
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].
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].
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].
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].
Takeuchi T, Kamide R. Severe hydroa vacciniforme-like eruptions confined to sun-exposed areas. J Dermatol. Apr 2011;38(4):386-9. [Medline].
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].
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].
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].
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].
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].
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].
Rhodes LE, White SI. Dietary fish oil as a photoprotective agent in hydroa vacciniforme. Br J Dermatol. Jan 1998;138(1):173-8. [Medline].
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].
Durbec F, Reguiaï Z, Léonard F, Pluot M, Bernard P. Efficacy of ?-3 Polyunsaturated Fatty Acids for the Treatment of Refractory Hydroa Vacciniforme. Pediatr Dermatol. Oct 4 2011;[Medline].
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].
Ashurst PJ. Hydroa vacciniforme occurring in association with Hartnup disease. Br J Dermatol. Jul 1969;81(7):486-92. [Medline].
Bickers DR, Demar LK, DeLeo V, Poh-Fitzpatrick MB, Aronberg JM, Harber LC. Hydroa vacciniforme. Arch Dermatol. Aug 1978;114(8):1193-6. [Medline].
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].
Ferguson J, Ibbotson S. The idiopathic photodermatoses. Semin Cutan Med Surg. Dec 1999;18(4):257-73. [Medline].
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].
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].
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].
Raza N, Bari AU, Farooq M. Hydroa vacciniforme in an adolescent girl. J Coll Physicians Surg Pak. Jun 2007;17(6):359-61. [Medline].
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].
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].
Taylor CR, Duke D. Blistering, scarring, and photosensitivity in a male teenager. Arch Dermatol. Oct 1999;135(10):1267, 1270. [Medline].
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].

Characteristic vesicular lesions occur on sun-exposed skin and heal with varioliform scarring.

View Table List

Read more about Hydroa Vacciniforme on Medscape

About Medscape Reference | Editorial & Advisory Board

[ CLOSE WINDOW ]

About Medscape Reference

Medscape Reference is the most authoritative and accessible point-of-care medical reference for physicians and health care professionals. All content is available free of charge, both online and via mobile devices.

Medscape Reference articles represent the expertise and practical knowledge of top physicians and pharmacists from leading academic medical centers in the US and worldwide. In addition, our rigorous literature survey process allows us to rapidly integrate new practice-changing information into the relevant topics by systematically reviewing the major medical and pharmacy journals, news announcements, and important practice guidelines.

Medscape Reference provides comprehensive coverage across more than 30 medical specialties and is composed of the following areas:

Diseases and Conditions

More than 6,300 evidence-based and physician-reviewed disease and condition articles are organized to rapidly and comprehensively answer clinical questions, as well as to provide in-depth information in support of diagnosis, treatment, and other clinical decision-making. Topics are richly illustrated with more than 30,000 clinical photos and radiographic images.

Procedures

More than 650 clinical procedure articles provide clear, step-by-step instructions, and include instructional videos and images to allow clinicians to master the newest techniques or to improve their skills in procedures they have previously performed.

Drugs

More than 2,100 monographs, including prescription and over-the-counter drugs, plus over 5,000 corresponding brand-name drugs, herbals, and supplements, are provided. Drug images and pricing are also included. In addition, a Drug Interaction Checker provides rapid access to tens of thousands of interactions between brand and generic drugs, over-the-counter drugs, and herbals and supplements.

Anatomy

More than 70 anatomy articles feature clinical images and diagrams of the major human body systems and organs. The articles assist with the understanding of the anatomy involved in treating specific conditions and performing procedures. They can also facilitate physician-patient discussions.

Special Features
Best Evidence

This email newsletter features current and clinically relevant journal citations that are selected via our rigorous literature survey review process. These same citations are incorporated into the relevant Medscape Reference articles. This newsletter serves as a quick and easily accessible way to stay up to date with clinically relevant journal articles that impact clinical practice.

Case Studies

These challenging and classic case presentations provide a quick way for practicing physicians to sharpen their diagnostic and patient-management skills. Each case starts with how the patient presented in the clinic and then using a question and answer format, progresses through diagnosis and on to treatment and includes follow up information about outcome for the patient.

Image collections

Image collections are a visually-engaging presentation of both common and uncommon diseases, case presentations, and current controversies in medicine. They are presented in an image rich, slideshow format and are designed to challenge and expand physicians' knowledge of clinically important topics.

Medscape is the leading online destination for healthcare professionals for clinical information. In addition to clinical reference tools, Medscape also offers:

Medscape News Learn more
Medscape Education Learn more