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A 15-year-old boy presented with vesiculopapular eruptions confined to sun-exposed areas from the age of 3. Histopathological examination of biopsy specimens of repetitive UVA-irradiated areas revealed reticular degeneration of the epidermis, and dermal infiltrates in the photoinduced lesions showed a latent Epstein-Barr virus (EBV) infection. At the age of 21, the number of skin lesions had increased, and his anti-EBV antibody titers revealed an abnormal profile: an undetectable anti-EBV nuclear antigen antibody titer despite a detectable antiviral capsid antigen IgG antibody titer. No infectious mononucleosis-like symptoms, such as prolonged or intermittent fever, lymphoadenopathy, or liver damage were evident up till then. Severe hydroa vacciniforme (HV)-like eruptions were diagnosed considering the increased number of the skin lesions with increasing age and the unusual anti-EBV antibody titers, in addition to the histopathological findings. In the same year, he suddenly developed high fever and died from disseminated intravascular coagulation syndrome without any spontaneous regression of the skin lesions. In this patient with severe HV-like eruptions, the skin lesions had been confined to sun-exposed areas until his death, and the photo-provocation test showed a positive reaction. Severe HV-like eruptions may have clinical features suggestive of HV., (© 2010 Japanese Dermatological Association.)

Photoallergic dermatoses are skin lesions following sun exposure, characterized by activation of immunological mechanisms, involving photosensitizers and photoallergens that can cause photosensibilization in some individuals. In this group of disorders, photoallergic contact dermatitis should be accentuated. It manifests as contact allergic dermatitis on sun-exposed skin areas, following direct contact with photoallergens during UV exposure (predominantly UVA). Under influence of light, photosensitizers get activated, followed by fusion with cutaneous proteins which renders them to complete antigens, and consequential initiation of immunological mechanisms with resulting pathological skin lesions. The most common photoallergens are: sulfonamide antibiotics, phenothiazines, and halogenated salicylanilides. Photoallergic dermatoses are comprised of several disorders, although the causative photoallergen remains unknown (e.g. solar urticaria, polymorphous light eruption and hydroa vacciniforme). Solar urticaria is a rare, acute urticarial reaction on both sun-exposed and covered skin areas, which appears soon after exposure to sun or artificial lighting. Polymorphous light eruption is a relatively common polymorphous skin eruption, which usually appears in spring. Its pathogenesis is unknown, presumably photoallergic reaction. Hydroa vacciniforme is a rare photodermatosis of unknown etiology, which usually presents in summer-time. It is characterized by vesicobullous eruptions, with residual nonesthetic varioliform scarring.

Background: Cases of hydroa vacciniforme-like eruptions caused by latent Epstein-Barr virus (EBV) infection have been reported in Asia and Mexico, some of which progressed to lymphoproliferative disorders. The precise clinical features of the disease are not, however, clear., Methods: We performed an artificial provocation test with repeated exposures of ultraviolet A (UVA) and UVB in an 8-year-old Korean girl who had severe hydroa vacciniforme-like skin eruptions on exposed areas., Results: The patient showed features of latent EBV infection serologically, and an in situ hybridization study indicated that most infiltrating mononuclear cells in the dermis were positive for EBV. After three exposures to UVA irradiation with a total dose of 90 J/cm2, erythema and vesicles appeared on the UVA-irradiated site but not on the UVB-irradiated site., Conclusions: Atypical hydroa vacciniforme caused by latent EBV infection could be reproduced by repeated UVA irradiation. Further study on the photo-protection would be necessary to prevent its progression to lymphoproliferative malignancies.

Hydroa vacciniforme (HV) is a rare blistering photodermatosis that heals with vacciniform scarring, with onset usually in childhood and spontaneous resolution by early adulthood. We report two cases of HV seen at the National Skin Centre, Singapore, both with a late onset at 20 years of age, during compulsory military service, and one with a less typical non-vesicular presentation, that initially caused some diagnostic difficulties.

Epstein Barr virus (EBV) infection is implicated in various kinds of neoplasms including certain types of cutaneous T or natural killer (NK) cell proliferative disorders. Although a pathogenic role of EBV infection is not clear, some EBV gene products expressed during a latency phase were found to have biological properties leading to cellular gene expression and immortalization. Furthermore, EBV can use an array of strategies to evade host immune responses, and maintain the latent infection. EBV-associated cutaneous lymphoproliferative disorders are prevalent in Asia, and less frequent in western countries where infectious mononucleosis is common in adolescents and young adults. This review introduces recent advances on the mechanism of EBV infection, highlighting unique clinicopathologic manifestations of EBV-associated cutaneous lymphoproliferative disorders.

Hydroa vacciniforme is a rare, idiopathic photodermatosis with an onset in childhood and characterized by acute vesiculation, crusting and scarring following sun exposure. Familial cases are extremely rare with only one previous report. We report a brother and sister, both of whom have developed hydroa vacciniforme.

Hydroa vacciniforme vesicles can be reproduced experimentally with repetitive UVA irradiations, but this photo-reproduction is not constant. The aim of this study was to search for the factors that influence photo-reproduction. To reproduce hydroa vacciniforme lesions six patients underwent repetitive UVA and polychromatic irradiations on the back. In four patients out of six, UVA irradiation with high doses induced papulo-vesicular lesions. Photo-induced lesions were very close to those induced by sunlight. Photo-reproduction failed when phototesting was done after or shortly before remission. Therefore, the absence of photo-reproduction appears to be a good prognostic factor.

Idiopathic polymorphous light eruptions (PLE) are photodermatites due to an as yet unidentified photosensitizing agent. Benign estival PLE is the most frequent form: the face is spared, and as soon as the patient has acquired a slight tan the eruption will no longer occur. Juvenile spring eruption affects the free border of the helix and may be regarded as a localized and mild form of estival PLE. Polymorphous light eruptions appear as lesions of the face which may resemble lupus erythematosus; photobiological exploration reproduces the lesions experimentally in 70% of the cases; immunohistochemistry studies are in favour of an immunological mechanism. Solar urticaria is a rare physical urticaria started by UVA, UVB and/or visible light: tolerance to sunlight can be induced by repeated exposures, but it does not last. Hydroa vacciniform is a rare PLE in children with leaves smallpox-like scars; photobiological exploration reproduces the lesions with high doses of UVA applied for 3 consecutive days. The remanent photosensitivity syndrome is characterized by an extreme photosensitivity which may be very disabling, preventing the patient to go outdoors; photo-allergological exploration discloses numerous positive patch-tests or photopatch-tests to various allergens, but the role played by these allergens in the occurrence of photosensitivity remains unclear. The syndrome probably results from an exogenous photosensitivity accident that has not regressed after exclusion of the presumably responsible photoallergens. Many treatments tested for these PLE seem to be effective, but they have not yet been controlled by double-blind trials.

The acute idiopathic photodermatoses are more common in females and comprise polymorphic light eruption, actinic prurigo, hydroa vacciniforme, and solar urticaria. Polymorphic light eruption occurs considerably more frequently than the others and while precise pathogenic mechanisms are still unclear, increasing evidence suggests an immunological basis for this condition. Although clinically distinct, actinic prurigo may be a variant of polymorphic light eruption, whereas solar urticaria and possibly hydroa vacciniforme are distinct entities, the former representing a type I hypersensitivity response. Polymorphic light eruption is characterized by a recurrent cutaneous reaction to ultraviolet (UV) exposure occurring after a delay of several hours that consists of pruritic erythematous papules, vesicles, or plaques on usually only some exposed sites and resolves without scarring over about a week. Actinic prurigo is differentiated from polymorphic light eruption by childhood onset and more persistent and excoriated lesions present both on sun-exposed and, to a lesser extent, non-exposed sites. Hydroa vacciniform is a rare disorder that also begins in childhood, and is characterized by recurrent crops of vesicles on sun-exposed skin and subsequent vacciniforme scarring. Solar urticaria is an uncommon condition that usually begins in the third or fourth decade and is differentiated from the other acute idiopathic photodermatoses by rapid onset of urticarial lesions within minutes of UV exposure and resolution within 1 to 2 hours.

Hydroa vacciniform is a rare recurrent photosensitive skin condition in which deep-seated vesicles appear on sun-exposed areas and heal leaving a depressed scar. Two cases are reported, with particular emphasis on phototesting investigations. Results of blood and urine porphyrin studies were normal and no systemic abnormalities were noted. Phototesting may be viewed as an important diagnostic aid, since the induction of lesions, clinically identical to hydroa vacciniform can occur following sequential exposure to UV-A, as was the case in one of our patients.

Action spectra for UV erythema and reproduction of the skin lesions in 4 patients with hydroa vacciniforme (HV) were studied. Vesicles were successfully reproduced by repeated daily monochromatic irradiations of 330, 340, 350 and 360 nm for 5, 6 or 7 days. The daily doses were equivalent to global solar radiation for 70-190 min. The vesicles produced were identical to those of HV, macroscopically and histologically. Repeated daily exposures to a small dose of UVA produced vesicles, whereas a large but single exposure to UVA failed to do so. Daily exposures to UVB failed to produce the vesicles. The minimal erythema dose (MED) was lower than the minimum limits of the normal persons in the 290-310 nm range.

Hydroa vacciniforme is a rare photosensitivity disorder with onset in childhood. The distinctive lesion is a vesicle which heals with scarring. We report a case of hydroa vacciniforme in which an abnormal minimal erythema dose to wavelengths of 322 to 370 nm within the ultraviolet A (UVA) range was demonstrated. Vesicles could be induced only with multiple exposures to UVA. Increased tolerance to UVA erythema was induced by multiple UVB exposures, although tanning was poor. Of note were the presence of several halo nevi and a history of the loss of ability to tan. The clinical features and appropriate laboratory evaluation of hydroa vacciniforme are reviewed.

The authors described three cases of Bazin's hydroa vacciniforme. Study of the history of the disease enables present nosological conceptions to be better understood. Diagnosis of hydroa vacciniforme is essentially clinical : appearance of the rash, periodicity and spontaneous improvement after puberty are characteristic. Hydroa vacciniforme has to be distinguished from porphyrias and polymorphous light sensitive eruptions. The etiology of this photodermatosis of child is unknown, but associated disturbances in tryptophan metabolism in some cases seem to be linked to a vitamin B6 deficiency.