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AT-A-GLANCE

AT-A-GLANCE

  • A rare, chronic, scarring photodermatosis sometimes associated with Epstein-Barr virus infection.

  • Characterized by recurrent sunlight-induced crops of papulovesicles and vesicles, most commonly on the face and dorsa of the hands.

  • Onset commonly in childhood, remitting most often at puberty.

  • May be a scarring variant of polymorphic light eruption.

  • Focal intraepidermal vesiculation, reticular degeneration of the epidermis, epidermal and upper dermal necrosis, and sometimes ulceration are typical histologic changes.

  • Avoidance of ultraviolet radiation including the use of broad-spectrum sunscreens is the only established therapy, but there may be a role for antivirals.

  • A severe, often fatal lymphoma resembling hydroa vacciniforme is distinguished by fever, facial edema, and systemic symptoms.

DEFINITION

Hydroa vacciniforme (HV) is a rare, chronic photodermatosis with onset typically occurring in childhood and a tendency to remission in adolescence. HV is characterized by photoinduced papules and vesicles that invariably scar after healing.

HISTORICAL PERSPECTIVE

HV was first described in 1862 by Bazin.

EPIDEMIOLOGY

HV is reported most often in North America, Europe, and Japan but is known to occur globally. The disease has its onset in childhood, most often presenting before age 8 years; presentation in adulthood is unusual. Patients with light pigmentation are affected preferentially. The disease is rare, with one estimate of the prevalence of HV being 0.34 cases per 100,000 individuals with an approximately equal sex ratio.1 There is male predominance for severe forms, whereas milder disease is more common in females.1,2 Cases of HV are normally sporadic, and familial incidence is exceptional.

CLINICAL FEATURES

HISTORY

HV commonly develops in early childhood and resolves spontaneously by puberty, although, in some patients, it is lifelong. HV eruptions typically occur in summer,3 often with an intense burning or stinging sensation followed by the appearance of individual or confluent papules and then vesicles, all within hours of sunlight exposure (Fig. 94-1). This is followed by umbilication, crusting, and progression to permanent pock scarring within weeks.

Figure 94-1

Hydroa vacciniforme. Vesicular, bullous, and crusted facial lesions, which heal with vacciniform scars.

CUTANEOUS FINDINGS

HV is characterized initially by erythema, sometimes with swelling, followed by the eruption of tender papules and vesicles within 24 hours of sun exposure. The eruption affects the cheeks and to a lesser extent other areas of the face, as well as the backs of the hands and dorsal aspects of the arms. The distribution tends to be symmetrical. Vesicles may occasionally become confluent and hemorrhagic. Later, papules and vesicles umbilicate and develop ulceration. Within weeks to months, lesions heal leaving permanent, depressed, hypopigmented scars. These scars are invariably present and resemble those seen in vaccinia (“pock ...

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