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Hydroa Vacciniforme Clinical Presentation

  • Author: Gregory Toy; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Jun 21, 2016
 

History

Commonly, mild burning, itching, or stinging in exposed sites begins a few hours or days after sun exposure. Vesicles heal with varioliform scarring.[3] 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.

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Physical

Skin and mucous membranes[8] are the primary sites affected by hydroa vacciniforme (HV). Ocular involvement is uncommon and usually occurs along with an HV outbreak of the face.[9]

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[9, 3] :

  • Mild keratoconjunctivitis
  • Corneal clouding and stellate keratotic precipitates in the cornea, indicating an inflammatory keratitis (one report[10] )
  • 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)[11]

Severe HV may present with the following[12] :

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

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.[13] Ultraviolet A (UV-A) radiation is most often implicated. Two reports of HV in siblings have been documented, suggesting a genetic component to HV.[14] Some cases have occurred in the setting of hematopoietic malignancy. Moreover, HV can be caused by a recent or latent EBV infection.[4]

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Contributor Information and Disclosures
Author

Gregory Toy State University of New York Downstate College of Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Marjon Vatanchi, MD Pediatric Dermatology Research Fellow, Department of Dermatology, SUNY Downstate Medical Center

Marjon Vatanchi, MD is a member of the following medical societies: American Academy of Dermatology, Houston Dermatological Society, Medical Dermatology Society, National Psoriasis Foundation, Texas Dermatological Society

Disclosure: Nothing to disclose.

 

Disclosure: Nothing to disclose.

Specialty Editor Board

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

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.

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