History

Commonly, mild burning, itching, or stinging in exposed sites begins a few hours or days after sun exposure. Vesicles heal with varioliform scarring.^[4]The initial onset of lesions occurs in spring, with recurrences in summer months.

Constitutional symptoms can occur but are uncommon. Oral and ocular symptoms can occur but are extremely rare.

Physical Examination

Skin and mucous membranes^[13]are the primary sites affected by hydroa vacciniforme (HV). Ocular involvement is uncommon and usually occurs along with an HV outbreak of the face.^[14]

Skin findings are as follows:

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.

Eye findings are as follows^{[14, 4]}:

Mild keratoconjunctivitis
Corneal clouding and stellate keratotic precipitates in the cornea, indicating an inflammatory keratitis (one report^[15])
Conjunctivitis and vesicular eruptions of the conjunctiva
Corneal infiltration with vascularization
Keratouveitis

Other symptoms are as follows:

Photo-onycholysis
Limited partial absorption of bone and cartilage in severe HV
Earlobe mutilation and flexion contracture of a digit (reported in 1 patient)^[16]

Severe HV may present with the following^[17]:

High-grade fever
Liver damage
Ulcerative indurated lesions
Edematous swelling of the cheeks, ears, lips, and eyelids

Complications

Complications are rare in hydroa vacciniforme (HV). The most common severe sequela is the varioliform scarring.

Differential Diagnoses

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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].
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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].
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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 Clinical Presentation

History

Physical Examination

Complications

References

Tables

Contributor Information and Disclosures

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