Hydroa Vacciniforme Clinical Presentation

Updated: Aug 05, 2019
  • Author: Gregory Toy; Chief Editor: Dirk M Elston, MD  more...
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Presentation

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.

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Physical Examination

Skin and mucous membranes [12] are the primary sites affected by hydroa vacciniforme (HV). Ocular involvement is uncommon and usually occurs along with an HV outbreak of the face. [13]

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 [13, 4] :

  • Mild keratoconjunctivitis

  • Corneal clouding and stellate keratotic precipitates in the cornea, indicating an inflammatory keratitis (one report [14] )

  • 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) [15]

Severe HV may present with the following [16] :

  • High-grade fever
  • Liver damage

  • Ulcerative indurated lesions

  • Edematous swelling of the cheeks, ears, lips, and eyelids

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Complications

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

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