Abstract
A 57-year-old man with no history of alcohol intake and drug inducing porphyria developed fragility, blistering, small scarring and hyperpigmentation on the skin of the face and backs of the hands. He had no episode of acute photosensitivity. He had a chronic, active hepatitis. There was an abnormal increase in 24hr urinary porphyrin excretion, therefore cutaneous lesions suggested hepatic cutaneous porphyria. In the histopathological examinations, deposition of PAS-positive materials was observed in the dermoepidermal basement membrane zone and small dermal blood vessel-wall of the scar-like eruption on the back of the hand. Direct immunofluorescence showed marked deposition of IgG and fibrinogen and mild deposition of IgA in the regions in which the PAS-positive materials were deposited. Similar findings were observed in the clinically normal skin not exposed to sunlight, but the magnitude of the deposition of IgG, IgA and fibrinogen was much less marked than that occurring in the cutaneous lesions exposed to sunlight.
