ARTICLE
Hydroa vacciniforme (HV) is an extremely rare chronic photodermatosis
of unknown origin, with onset in childhood and spontaneous resolution
in adolescence or early adulthood. The lesions consist of vesiculopapules
with epidermal necrosis, and resolve invariably leaving varioliform scars
[1, 2]. The course of HV is characterized by recurrent flares upon sun
exposure, and, in many cases, lesions can be reproduced with repeated
exposures to UVA, or less frequently, to UVB [3-6]. We describe a patient
with typical HV in whom lesions began in childhood and continued until
60 years of age.
Case report
A 60-year-old Caucasian man presented in April 1997 with erythema, papulovesicles,
yellowish crusts and scars on his ears and preauricular areas (Fig.
1). Since the age of 8, the patient had suffered from recurrent
episodes of erythematous macules, papules, vesicles, erosions and ulcers
on the same sites, as well as on the nose and cheeks, gradually resulting
in permanent scars. Lesions generally occurred in crops about 1-2 days
after sun exposure, from March to October. Nail changes or eye involvement
never occurred. The histological changes consisted of epidermal vesiculation
and necrosis, and perivascular dermal infiltrates of neutrophils and mononuclear
cells. No ballooning degeneration of epidermal
cells or multinucleated giant cells could be detected. There was no family
history of skin disease. The patient took no medication. Routine blood
tests as well as blood, urine and stool porphyrin concentration, and urinary
amino acid levels were within normal limits. Circulating antinuclear,
anti-SSA and anti-SSB antibodies were absent. Direct immunofluorescence
performed on both affected and normal sun-exposed skin was negative. The
minimal erythema dose (MED) for both UVA and UVB was within normal range
for the patient's skin type, and no lesions appeared on the back skin
after repeated irradiation with UVA or UVB (1-5x MED for 4 days), even
if phototesting was performed during the active period of the disease
[6]. Photopatch tests with a standard series of photoallergens were also
negative. Avoidance of sun exposure, the use of broad spectrum sunscreens,
and treatment with hydroxychloroquine (200 mg/day) resulted in lesion
resolution within 30 days.
Discussion
HV usually begins in childhood and resolves by early adult life. Lesions
of HV may appear on any site exposed to sunlight, although helices of
the ears, cheeks, and the bridge of the nose are most commonly affected.
In the majority of patients, lesions appear in crops during early spring
and fade in fall, but winter sunlight can trigger an HV attack. Some patients
with HV also manifest photo-onycholysis, and ocular involvement with conjunctivitis
and/or keratitis [1, 2]. Skin lesions start as itchy red macules from
15 min to 24 hrs after sunlight exposure. Macules evolve into tender and
painful red papules, that progress into tense vesicles and sometimes in
bullae that umbilicate, become flaccid, hemorrhagic centrally, and are
then followed by crust formation. Finally, lesions leave hypopigmented
scars. Histological findings are quite distinctive, although they are
not pathognomonic. The hallmark is vesicles due to epidermal necrosis,
and dermal edema with a mixed infiltrate consisting of mononuclear cells,
neutrophils and eosinophils. In the differential diagnosis of HV, other
sunlight triggered dermatoses should be considered, including porphyrias,
polymorphous light eruption, drug photosensitivity, photocontact dermatitis,
discoid lupus erythematosus, herpes simplex, and especially hydroa aestivale.
In this latter condition, there is no epidermal necrosis and the lesions
resolve without scarring. However, some authors consider HV as a more
severe form of hydroa aestivale. Pathogenesis of HV is still unknown.
In many patients, lesions can be reproduced on normal skin by repeated
irradiation with UVA, or, less frequently, with UVB. However, the MED
was low in some patients but normal in others [3-6]. In our patient, the
MED was within normal limits, and we could not induce any lesions on normal
skin with multiple UVA or UVB irradiation. Treatment
is often unsatisfactory. Topical wide spectrum sunscreens, and hydroxychloroquine,
as demonstrated in our patient, may be helpful. Other approaches include
carotenoids, polyunsaturated fatty acids, or, in more severe cases, azathioprine
[7, 8]. Finally, prophylactic phototherapy with UVA or narrow-band UVB
may be of benefit in some cases [9]. Although HV generally resolves spontaneously
in early adulthood, in our patient it was still present at the age of
60. Only very few cases of HV have been previously reported to persist
in adult life. McGrae and Perry noted the persistence of symptoms into
the fourth decade in three of their 29 cases, but they did not exclude
proto-porphyria [1]. In addition, a man suffering from HV since the age
of 19 and permanently cured at the age of 47 following chemotherapy for
Hodgkin's disease, has been described [10].
REFERENCES
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induction of lesions with ultraviolet A. J Am Acad Dermatol 1983;
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4. Eramo LR, Garden JM, Esterly NB. Hydroa vacciniforme, diagnosis by
repetitive ultraviolet-A phototesting. Arch Dermatol 1986; 122:
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hydroa vacciniforme. Br J Dermatol 1998; 138: 173-8.
9. Collins P, Ferguson J. Narrow-band UVB (TL-01) phototherapy: an effective
preventive treatment for the photodermatoses. Br J Dermatol 1995;
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10. Steger GG, Dittrich C, Hönnigsmann H, Moser K. Permanent cure
of hydroa vacciniforme after chemoterapy for Hodgkin's disease. Br
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