Australas J. Dermatol 1992; 33: 118-120
AUSTRALIAN DERMATOPATHOLOGY SOCIETY RECURRENT VESICULAR LESIONS ON SUN EXPOSED SKIN VANESSA A. MORGAN, GEORGE A. VARIGOS AND C. W. CHOW
Royal Children's Hospital, Melbourne A boy aged six and a half years presented to The Royal Children's Hospital with a one year history of recurrent vesicular lesions on sun exposed skin - face, neck, ears and dorsal surfaces of hands. (Figs. 1 & 2) Attacks had occurred following light exposure through window glass, and short exposure to light was followed by stinging and burning without progression to blisters. On four occasions severe attacks had occurred: within 24 hours of sun exposure blisters developed. Subsequently the blisters became haemorrhagic and developed crusts. (Fig. 3) Healing took two to four weeks, leaving varioliform scars. He was otherwise well, with no photophobia or abdominal or joint pain. There was no past history of skin disease, and he had not been exposed to any photosensitizing drugs or external agents. He had no siblings, and his parents had not been affected by any skin disease. His general development was normal. On examination he was a well looking, bright boy with fair skin and hair and blue eyes. His vital Presented by Vanessa A. Morgan, George A. Varigos and C.W. Chow, Royal Children's Hospital, Melbourne.
signs were stable and he was on the 95th percentile for height and weight. He had flaccid blisters on the face, ears, back of neck, dorsum of hands and lips, with central haemorrhage, crusting and necrosis. There were no lesions in the mouth and the rest of the examination was unremarkable. The patient had been treated by the local medical officer with broad spectrum topical sunscreen, topical and oral acyclovir (herpes simplex culture negative) prior to referral, without significant effect. Full blood examination, urea and electrolytes, liver function tests and urine, faecal and red blood cell porphyrins were shown to be within normal limits. Anti nuclear factor (ANF) and urine metabolic screens were negative. He had an elevated erythrocyte sedimentation rate of 30. A skin biopsy revealed epidermal necrosis with blister formation (Fig. 4, H&E, XI3.2). The slides illustrate these features, with a mixed inflammatory cell infiltrate which hugs the epidermis, and some exocytosis of inflammatory cells into the epidermis. Red cells, neutrophils and lymphocytes can be seen within the vesicles (Fig. 5, H&E, X33). In addition there was marked spongiosis of the adjacent non-necrotic epidermis.
FIGURE 1
FIGURE 2
118
VANESSA A. MORGAN, GEORGE A. VARIGOS AND C. W. CHOW
RECURRENT VESICULAR LESIONS ON SUN EXPOSED SKIN
HYDROA VACCINIFORME
This is a rare photodermatosis of unknown aetiology first described by Bazin in 1862. There is some controversy regarding the wavelength of light responsible but it is most probably UVA induced. Hydroa vacciniforme has the following features, most of which are illustrated by this case: 1. Onset in childhood with males affected twice as often as females. A familial association has been reported in three patients.' 2. Lesions develop several hours, or up to one to two days after sun exposure. They begin as vesicles or bullae on an erythematous base which undergo central haemorrhage, crusting and necrosis before eventually healing with varioliform scars. In addition, they may be preceded by burning or stinging; there may be mild constitutional disturbance; 10% develop eye involvement - usually photophobia and mild keratoconjunctivitis, although anterior uveitis, which may lead to loss of vision if not detected, has been reported;^ fair, easily sunburned skin would appear to be most commonly affected;' there has been one case in association with Hartnup disease.'' Hydroa vacciniforme usually subsides in late teenage years but may continue to adult life. 3. There is a characteristic histopathology with epidermal necrosis and intraepidermal vesiculation. Direct immunofluorescence is negative. 4. There are no specific laboratory abnormalities, including serological and porphyrin studies. Low complement, however, has been reported.' The patient's elevated ESR is a non specific finding. 5. Phototesting results have been inconsistent but it has been suggested that this is because multiple exposure is needed to induce a response. One group suggest that repetitive UVA, 40 joules/square cm in two 20 joule doses is a useful diagnostic aid.'
120
In this case the diagnosis was definite, with the combination of the clinical, pathological and laboratory features. The differential diagnosis includes porphyria (differentiate on the basis of the family history, porphyrin studies and histology), lupus erythematosus (differentiate on the basis of positive serological profile and on history), polymorphic light reaction (differentiate on the basis of later onset, rare vesiculation, no scars and no epidermal necrosis), drug photosensitivity and a miscellaneous group of conditions including varicella, herpes simples, Hartnup disease, Hutchinson's spring eruption, bullous lichen planus, Darier's disease, photosensitive atopic eczema and pellagra. Investigations necessary to make a diagnosis therefore include a skin biopsy, porphyrin studies, ANF and a urine metabolic screen to exclude Hartnup's disease. The management of these patients is difficult as protection by broad spectrum sunscreens is often ineffective. Sun avoidance is essential and an ophthalmologist should be consulted. Antimalarials, B carotene, UVB, PUVA, vitamin B6 and thalidomide have been tried with variable results. When outdoors this patient wears a plastic helmet, made of a material which screens out 95% of UVA. He has been treated with B carotene 30mg tds and an antihistamine as required for acute episodes. REFERENCES ' Annamali R. Hydroa vacciniforme in three alternate siblings. Arch Dermatol 1971; 103: 224-225. ' Bennion SD, Johnson C, Weston WL. Hydroa vacciniforme with inflammatory keratitis and secondary uveitis. Pediatr Dermatol 1987; 4: 320-324. ' Sonnex TS, Hawk JL. Hydroa vacciniforme: a review of ten cases. Br J Dermatol 1988; 118: 101-108. ' Ashurst PJ. Hydroa vacciniforme occurring in association with Hartnup disease. Br J Dermatol 1969; 81: 486-491. ' Goldgeier MH, Nordlund JJ, Lucky AW, Sibrack LA, McCarthy MJ, McGuire J. Hydroa vacciniforme. Arch Dermatol 1982; 118: 588-591. ' Eramo LR, Garden JM, Esterly NB. Hydroa vacciniforme: diagnosis by repetitive ultraviolet-A phototesting. Arch Dermatol 1986; 122: 1310-1313.
AUSTRALIAN DERMATOPATHOLOGY SOCIETY RECURRENT VESICULAR LESIONS ON SUN EXPOSED SKIN VANESSA A. MORGAN, GEORGE A. VARIGOS AND C. W. CHOW
Royal Children's Hospital, Melbourne A boy aged six and a half years presented to The Royal Children's Hospital with a one year history of recurrent vesicular lesions on sun exposed skin - face, neck, ears and dorsal surfaces of hands. (Figs. 1 & 2) Attacks had occurred following light exposure through window glass, and short exposure to light was followed by stinging and burning without progression to blisters. On four occasions severe attacks had occurred: within 24 hours of sun exposure blisters developed. Subsequently the blisters became haemorrhagic and developed crusts. (Fig. 3) Healing took two to four weeks, leaving varioliform scars. He was otherwise well, with no photophobia or abdominal or joint pain. There was no past history of skin disease, and he had not been exposed to any photosensitizing drugs or external agents. He had no siblings, and his parents had not been affected by any skin disease. His general development was normal. On examination he was a well looking, bright boy with fair skin and hair and blue eyes. His vital Presented by Vanessa A. Morgan, George A. Varigos and C.W. Chow, Royal Children's Hospital, Melbourne.
signs were stable and he was on the 95th percentile for height and weight. He had flaccid blisters on the face, ears, back of neck, dorsum of hands and lips, with central haemorrhage, crusting and necrosis. There were no lesions in the mouth and the rest of the examination was unremarkable. The patient had been treated by the local medical officer with broad spectrum topical sunscreen, topical and oral acyclovir (herpes simplex culture negative) prior to referral, without significant effect. Full blood examination, urea and electrolytes, liver function tests and urine, faecal and red blood cell porphyrins were shown to be within normal limits. Anti nuclear factor (ANF) and urine metabolic screens were negative. He had an elevated erythrocyte sedimentation rate of 30. A skin biopsy revealed epidermal necrosis with blister formation (Fig. 4, H&E, XI3.2). The slides illustrate these features, with a mixed inflammatory cell infiltrate which hugs the epidermis, and some exocytosis of inflammatory cells into the epidermis. Red cells, neutrophils and lymphocytes can be seen within the vesicles (Fig. 5, H&E, X33). In addition there was marked spongiosis of the adjacent non-necrotic epidermis.
FIGURE 1
FIGURE 2
118
VANESSA A. MORGAN, GEORGE A. VARIGOS AND C. W. CHOW
RECURRENT VESICULAR LESIONS ON SUN EXPOSED SKIN
HYDROA VACCINIFORME
This is a rare photodermatosis of unknown aetiology first described by Bazin in 1862. There is some controversy regarding the wavelength of light responsible but it is most probably UVA induced. Hydroa vacciniforme has the following features, most of which are illustrated by this case: 1. Onset in childhood with males affected twice as often as females. A familial association has been reported in three patients.' 2. Lesions develop several hours, or up to one to two days after sun exposure. They begin as vesicles or bullae on an erythematous base which undergo central haemorrhage, crusting and necrosis before eventually healing with varioliform scars. In addition, they may be preceded by burning or stinging; there may be mild constitutional disturbance; 10% develop eye involvement - usually photophobia and mild keratoconjunctivitis, although anterior uveitis, which may lead to loss of vision if not detected, has been reported;^ fair, easily sunburned skin would appear to be most commonly affected;' there has been one case in association with Hartnup disease.'' Hydroa vacciniforme usually subsides in late teenage years but may continue to adult life. 3. There is a characteristic histopathology with epidermal necrosis and intraepidermal vesiculation. Direct immunofluorescence is negative. 4. There are no specific laboratory abnormalities, including serological and porphyrin studies. Low complement, however, has been reported.' The patient's elevated ESR is a non specific finding. 5. Phototesting results have been inconsistent but it has been suggested that this is because multiple exposure is needed to induce a response. One group suggest that repetitive UVA, 40 joules/square cm in two 20 joule doses is a useful diagnostic aid.'
120
In this case the diagnosis was definite, with the combination of the clinical, pathological and laboratory features. The differential diagnosis includes porphyria (differentiate on the basis of the family history, porphyrin studies and histology), lupus erythematosus (differentiate on the basis of positive serological profile and on history), polymorphic light reaction (differentiate on the basis of later onset, rare vesiculation, no scars and no epidermal necrosis), drug photosensitivity and a miscellaneous group of conditions including varicella, herpes simples, Hartnup disease, Hutchinson's spring eruption, bullous lichen planus, Darier's disease, photosensitive atopic eczema and pellagra. Investigations necessary to make a diagnosis therefore include a skin biopsy, porphyrin studies, ANF and a urine metabolic screen to exclude Hartnup's disease. The management of these patients is difficult as protection by broad spectrum sunscreens is often ineffective. Sun avoidance is essential and an ophthalmologist should be consulted. Antimalarials, B carotene, UVB, PUVA, vitamin B6 and thalidomide have been tried with variable results. When outdoors this patient wears a plastic helmet, made of a material which screens out 95% of UVA. He has been treated with B carotene 30mg tds and an antihistamine as required for acute episodes. REFERENCES ' Annamali R. Hydroa vacciniforme in three alternate siblings. Arch Dermatol 1971; 103: 224-225. ' Bennion SD, Johnson C, Weston WL. Hydroa vacciniforme with inflammatory keratitis and secondary uveitis. Pediatr Dermatol 1987; 4: 320-324. ' Sonnex TS, Hawk JL. Hydroa vacciniforme: a review of ten cases. Br J Dermatol 1988; 118: 101-108. ' Ashurst PJ. Hydroa vacciniforme occurring in association with Hartnup disease. Br J Dermatol 1969; 81: 486-491. ' Goldgeier MH, Nordlund JJ, Lucky AW, Sibrack LA, McCarthy MJ, McGuire J. Hydroa vacciniforme. Arch Dermatol 1982; 118: 588-591. ' Eramo LR, Garden JM, Esterly NB. Hydroa vacciniforme: diagnosis by repetitive ultraviolet-A phototesting. Arch Dermatol 1986; 122: 1310-1313.
