Sections

Overview

Background

Hydroa vacciniforme (HV) is a rare, chronic photodermatosis of unknown origin occurring in childhood that exists within the spectrum of Epstein-Barr virus–related lymphoproliferative disorders. These range from classic HV to fatal lymphoma.^[1]

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.^[2]Most cases remit spontaneously by late adolescence.^[3]See the image below.

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

Pathophysiology

The etiology of hydroa vacciniforme (HV) is uncertain. 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. There is an association between HV occurrence and latent Epstein-Barr virus (EBV) infection, particularly if necrotic lesions are present.^[4]T cells positive for EBV-encoded small nuclear RNA (EBER) have been detected in the cutaneous infiltrates of some patients with HV. Ultraviolet-induced cutaneous lesions with histopathology consistent with HV have also been shown to contain EBER-positive cells.^[5]In general, the EBV DNA blood load is higher in patients with HV than in patients with other photosensitivity disorders.^[6]

Etiology

The development of hydroa vacciniforme (HV) lesions and their distribution suggest a causal relationship between HV and ultraviolet (UV) exposure, although the pathogenetic mechanism remains unknown.^[7]Ultraviolet A (UV-A) radiation is most often implicated, although some older studies suggest ultraviolet B (UV-B) as the causal agent.^[8]Two reports of HV in siblings have been documented, suggesting a genetic component to HV.^[9]Some cases have occurred in the setting of hematopoietic malignancy. Moreover, HV can be caused by a recent or latent EBV infection.^[5]

Frequency

The frequency of HV varies according to country but is estimated to be around 0.34 cases per 100,000 people.

Sex

Males have a higher incidence of HV than females. Males who are affected may also have a longer course of disease than females.^[10]

Age

HV predominately affects children aged 3-16 years.^[2]In many cases, there is a bimodal age distribution of early childhood (age 1-7 y) and around puberty (age 12-16 y).^[8]The mean duration from onset of symptoms to resolution is 9 years. Resolution of symptoms typically occurs in adolescence or young adulthood, although symptoms persist throughout life in some patients.^[10]Cases of HV in infants and elderly persons have also been described.^[11]

Ethnicity

Asians and Latin Americans are more likely to develop severe HV, which includes facial edema, multiple vesicles, and extensive scarring in both sun-exposed and sun-protected skin.^[1]

Prognosis

The prognosis is uncertain; classic hydroa vacciniforme (HV) typically remits by adolescence. No mortality is associated with classic HV, but those with lymphoid atypia, rimming of lipocytes, and Amerindian heritage are at significant risk of progression and death. Classic chemotherapy is associated with adverse outcomes.^[12]

Patient Education

Patients are advised regarding strict sun avoidance, frequent application of high SPF sunscreens with UV-A blocking agents, and protective clothing.

Clinical Presentation

Quintanilla-Martinez L, Fend F. Deciphering hydroa vacciniforme. Blood. 2019 Jun 27. 133 (26):2735-2737. [QxMD MEDLINE Link].
Pahlow Mose A, Fisker N, Clemmensen O, Bygum A. Antiviral treatment of a boy with EBV-associated hydroa vacciniforme. BMJ Case Rep. 2014 Nov 24. 2014:[QxMD MEDLINE Link].
Almeida HL Jr, Kopp J, Jorge VM, Sartori DS, Velloso CD. Extensive hydroa vacciniforme. An Bras Dermatol. 2013 Aug. 88(4):620-2. [QxMD MEDLINE Link]. [Full Text].
Greywal T, Ly M, Wang A, Friedlander SF. Hydroa vacciniforme and granulomatous uveitis in association with EBV. J Am Acad Dermatol. May 2016. 74:AB226.
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. 2006 May. 142(5):587-95. [QxMD MEDLINE Link].
Verneuil L, Gouarin S, Comoz F, Agbalika F, Creveuil C, Varna M, et al. Epstein-Barr virus involvement in the pathogenesis of hydroa vacciniforme: an assessment of seven adult patients with long-term follow-up. Br J Dermatol. 2010 Jul. 163 (1):174-82. [QxMD MEDLINE Link].
Halasz CL, Leach EE, Walther RR, Poh-Fitzpatrick MB. Hydroa vacciniforme: induction of lesions with ultraviolet A. J Am Acad Dermatol. 1983 Feb. 8(2):171-6. [QxMD MEDLINE Link].
Chen CC, Chang KC, Medeiros LJ, Lee JY. Hydroa Vacciniforme and Hydroa Vacciniforme-Like Lymphoproliferative Disorder: A Spectrum of Disease Phenotypes Associated with Ultraviolet Irradiation and Chronic Epstein-Barr Virus Infection. Int J Mol Sci. 2020 Dec 7. 21 (23):[QxMD MEDLINE Link].
Annamalai R. Hydroa vacciniforme in three alternate siblings. Arch Dermatol. 1971 Feb. 103(2):224-5. [QxMD MEDLINE Link].
Gupta G, Man I, Kemmett D. Hydroa vacciniforme: A clinical and follow-up study of 17 cases. J Am Acad Dermatol. 2000 Feb. 42 (2 Pt 1):208-13. [QxMD MEDLINE Link].
Nomura H, Suzuki H, Egami S, Yokoyama T, Sugiura M, Tomita K, et al. A patient with elderly-onset atypical hydroa vacciniforme with an indolent clinical course. Br J Dermatol. 2015 Sep. 173 (3):801-5. [QxMD MEDLINE Link].
Cohen JI, Manoli I, Dowdell K, Krogmann TA, Tamura D, Radecki P, et al. Hydroa vacciniforme-like lymphoproliferative disorder: an EBV disease with a low risk of systemic illness in whites. Blood. 2019 Jun 27. 133 (26):2753-2764. [QxMD MEDLINE Link].
Yesudian PD, Sharpe GR. Hydroa vacciniforme with oral mucosal involvement. Pediatr Dermatol. 2004 Sep-Oct. 21(5):555-7. [QxMD MEDLINE Link].
Jeng BH, Margolis TP, Chandra NS, McCalmont TH. Ocular findings as a presenting sign of hydroa vacciniforme. Br J Ophthalmol. 2004 Nov. 88(11):1478-9. [QxMD MEDLINE Link].
Wisuthsarewong W, Leenutaphong V, Viravan S. Hydroa vacciniforme with ocular involvement. J Med Assoc Thai. 1998 Oct. 81(10):807-11. [QxMD MEDLINE Link].
Kim WS, Yeo UC, Chun HS, Lee ES. A case of hydroa vacciniforme with unusual ear mutilation. Clin Exp Dermatol. 1998 Mar. 23(2):70-2. [QxMD MEDLINE Link].
Haddad JM, Monroe HR, Hardin J, Diwan AH, Hsu S. Hydroa vacciniforme: a rare photodermatosis. Dermatol Online J. 2014 Aug 17. 20 (8):[QxMD MEDLINE Link].
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. 1999 Feb. 40(2 Pt 1):283-4. [QxMD MEDLINE Link].
Iwatsuki K, Ohtsuka M, Harada H, Han G, Kaneko F. Clinicopathologic manifestations of Epstein-Barr virus-associated cutaneous lymphoproliferative disorders. Arch Dermatol. 1997 Sep. 133(9):1081-6. [QxMD MEDLINE Link].
Hirai Y, Yamamoto T, Kimura H, Ito Y, Tsuji K, Miyake T, et al. Hydroa vacciniforme is associated with increased numbers of Epstein-Barr virus-infected γδdT cells. J Invest Dermatol. 2012 May. 132(5):1401-8. [QxMD MEDLINE Link].
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. 1998 Apr. 38(4):574-9. [QxMD MEDLINE Link].
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. 1998 May. 138(5):905-6. [QxMD MEDLINE Link].
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. 2007 Feb. 68(2):403-7. [QxMD MEDLINE Link].
Takeuchi T, Kamide R. Severe hydroa vacciniforme-like eruptions confined to sun-exposed areas. J Dermatol. 2011 Apr. 38(4):386-9. [QxMD MEDLINE Link].
Wang M, Wang S, Yang QP, Liu YM, Gao LM, Sun H, et al. Hydroa vacciniforme-like lymphoma of an adult: a case report with review of the literature. Diagn Pathol. 2013 May 1. 8:72. [QxMD MEDLINE Link]. [Full Text].
Sangueza M, Plaza JA. Hydroa vacciniforme-like cutaneous T-cell lymphoma: clinicopathologic and immunohistochemical study of 12 cases. J Am Acad Dermatol. 2013 Jul. 69(1):112-9. [QxMD MEDLINE Link].
Uchiyama M, Tsuboi R, Kusunoki T. A case of recurrent facial herpes simplex mimicking hydroa vacciniforme. Int J Dermatol. 2015 Mar. 54 (3):e84-5. [QxMD MEDLINE Link].
Magaña M, Massone C, Magaña P, Cerroni L. Clinicopathologic Features of Hydroa Vacciniforme-Like Lymphoma: A Series of 9 Patients. Am J Dermatopathol. 2015 Sep 10. [QxMD MEDLINE Link].
Miyake T, Iwatsuki K, Hirai Y, Yamamoto T, Hamada T, Fujii K, et al. The aim of the measurement of Epstein-Barr virus DNA in hydroa vacciniforme and hypersensitivity to mosquito bites. J Med Virol. 2020 Apr 7. [QxMD MEDLINE Link].
Mehta RV, Shenoi SD, Balachandran C, Pai S. Minimal erythema response (MED) to solar simulated irradiation in normal Indian skin. Indian J Dermatol Venereol Leprol. 2004 Sep-Oct. 70 (5):277-9. [QxMD MEDLINE Link].
Levoska MA, Cohen JI, Manoli I, Richard Lee CC, Ching SST, Shand J, et al. Recurrent scarring papulovesicular lesions on sun-exposed skin in a 22-year-old man. J Am Acad Dermatol. 2018 Mar. 78 (3):637-642. [QxMD MEDLINE Link].
Collins P, Ferguson J. Narrow-band UVB (TL-01) phototherapy: an effective preventative treatment for the photodermatoses. Br J Dermatol. 1995 Jun. 132(6):956-63. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Ziering CL, Rabinowitz LG, Esterly NB. Antimalarials for children: indications, toxicities, and guidelines. J Am Acad Dermatol. 1993 May. 28(5 Pt 1):764-70. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. 1996 May 2. 334(18):1150-5. [QxMD MEDLINE Link].
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. 1994 Apr 14. 330(15):1029-35. [QxMD MEDLINE Link].
Blackwell V, McGregor JM, Hawk JL. Hydroa vacciniforme presenting in an adult successfully treated with cyclosporin A. Clin Exp Dermatol. 1998 Mar. 23(2):73-6. [QxMD MEDLINE Link].
Rhodes LE, White SI. Dietary fish oil as a photoprotective agent in hydroa vacciniforme. Br J Dermatol. 1998 Jan. 138(1):173-8. [QxMD MEDLINE Link].
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. 1995 Oct. 105(4):532-5. [QxMD MEDLINE Link].
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. 2011 Oct 4. [QxMD MEDLINE Link].

Media Gallery

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

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Author

Gregory Toy, MD Resident Physician, Department of Internal Medicine, University of Utah School of Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Marjon Vatanchi, MD Board Certified Dermatologist, Board Certified Micrographic Dermatologic Surgeon

Marjon Vatanchi, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for Laser Medicine and Surgery, Dermatologic Society of Greater New York, Medical Dermatology Society, National Psoriasis Foundation, New England Dermatological Society, Society for Pediatric Dermatology, Women's Dermatologic Society

Disclosure: Nothing to disclose.

Jeannette Rachel Jakus, MD, MBA Clinical Assistant Professor, Director of Clinical Research, Assistant Program Director, Department of Dermatology, SUNY Downstate Medical Center; Dermatologist, Brody Dermatology

Jeannette Rachel Jakus, MD, MBA is a member of the following medical societies: American Academy of Dermatology, American Academy of Pediatrics, American Skin Association, Society for Pediatric Dermatology, Women's Dermatologic Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael J Wells, MD, FAAD Dermatologic/Mohs Surgeon, The Surgery Center at Plano Dermatology

Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, 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

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

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

Disclosure: Nothing to disclose.

Additional Contributors

Maureen B Poh-Fitzpatrick, MD Professor Emerita of Dermatology and Special Lecturer, Columbia University College of Physicians and Surgeons

Maureen B Poh-Fitzpatrick, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, New York Academy of Medicine, New York Dermatological Society

Disclosure: Nothing to disclose.

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, American Society for Laser Medicine and Surgery

Disclosure: Nothing to disclose.

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.

Hydroa Vacciniforme

Background

Pathophysiology

Etiology

Epidemiology

Frequency

Sex

Age

Ethnicity

Prognosis

Patient Education

References

Tables

Contributor Information and Disclosures

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