Effects of phlebotomy on urinary porphyrin pattern and liver histology in patients with porphyria cutanea tarda.

Urinary porphyrin profiles and liver histology have been investigated in a group of adult alcoholics with porphyria cutanea tarda (PCT) before and after one year phlebotomy. Both parameters were evaluated during the same period in a group of patients who did not undergo specific therapy for PCT. All patients were advised to abstain from alcohol. At the end of the one year observation period there was a significant fall of urinary total porphyrins and in the uro/coproporphyrin ratio in treated patients compared to basal values whereas no changes were found in controls. Liver biopsy findings revealed a significant reduction of hepatic fatty degeneration and siderosis with no changes in inflammatory infiltrates and fibrosis in treated patients, so the progression of liver disease was similar to controls. These results show that clinical and biochemical remission of PCT can occur independently of the evolution of the concomitant liver disease.

A clinical contribution to guideline criteria in the excision of pigmentary lesions.

The clinical diagnosis of cutaneous melanoma is often difficult at the first stages of the malignant proliferation. The proposed anamnestic and clinical guidelines can offer useful criteria for the majority of the cases but sometimes a preventive excision is required for lesions that only partially fulfill the classic markers of "at risk" lesions. In this paper based on a comparative study between 2000 pigmented lesions and 40 melanomas of uncertain clinical diagnosis, the Authors recognize 5 statistically significant clinical pattern of benign pigmented lesions undistinguishable from clinically unusual melanomas. Epidemiological data about sex and age of the patients, histologically classification, site of the pigmented lesions and malignant transformation of congenital nevi are also reported.

Hydroxychloroquine versus phlebotomy in the treatment of porphyria cutanea tarda.

Hydroxychloroquine and phlebotomy were compared in the treatment of porphyria cutanea tarda (PCT). Thirty patients received hydroxychloroquine (200 mg twice weekly) for 1 year and thirty-one underwent twice-monthly phlebotomies of 400 ml whole blood each, also for 1 year. Clinical signs of disease improved equally in both groups. At the end of the year, urinary porphyrin excretion had significantly improved in twenty-two out of thirty hydroxychloroquine-treated subjects, but in only eight out of the thirty-one patients who received phlebotomy. Liver histology showed significant regression of steatosis and siderosis in both groups compared with the pretrial biopsy, but the activity of liver disease, as judged by the extent of necrosis, inflammation and fibrosis, worsened in twelve hydroxychloroquine and in seven phlebotomy-treated patients. It is concluded that hydroxychloroquine is more effective than phlebotomy in decreasing porphyrin production. However, further work is needed to assess whether long-term hydroxychloroquine treatment favours the progression of the chronic liver disease associated with PCT.