Eight men with porphyria cutanea tarda (PCT) ranged in age from 41 to 72 years and had a history of excessive alcohol intake. Distribution of the patients was as follows: six in Nagasaki (one in Nagasaki city, one in Sasebo city, 2 in Higashisonogi county, 2 in Kitamatsuura county), one in Fukuoka, one in Saga. Hyperpigmentation on exposed areas was present in all patients. Small scars, vesicles and erosions were seen in most patients, however, acute photosensitivity was evident only in two. Cutaneous changes were to a lesser degree those than described in previous reports. Urinary porphyrin analysis showed an increase of uroporphyrin (UP) and coproporphyrin (CP) levels and a predominance of UP in 7 of 8 cases. In one, there was an equal excretion of UP and CP in the urine. In the fecal porphyrin analysis, a predominance of CP excretion was seen in six of six patients. The fecal CP/PP ratio was reversed in the patients with PCT, compared to that in normal controls. Slight, abnormalities in liver function were noted in 7 of 8 patients, mainly slight increase of serum GOT, GPT and
γ-GTP levels. Liver biopsy was performed in 5 and the histopathology showed evidence of chronic hepatitis. Deposition of iron granules was evident in 4 of 5. Serum iron levels were increased to over 200
μg/dl in 4 of 8. A decrease of unsaturated iron binding capacity was seen in 4 of 8. There was an increase of serum IgG levels over 2,000 mg/dl in 3. From these results, our patients had mild cutaneous changes and slight biochemical abnormalties. It is suggested that cutaneous changes were influenced by the extent sun exposure time, without any correlation between the urinary porphyrin levels and the severity of the cutaneous symptoms.
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