Cutaneous Hepatic Porphyria and Oestrogens
Authors: P. W. M. Copeman, D. J. Cripps, R. Summerly
Journal: BMJ (1966)

Study of the response of the various porphyrias to drugs contributes to the understanding of their dissimilar biochemistry and pathogenesis (De Matteis and Rimington, 1962; Rimington, 1964).The cutaneous hepatic porphyria that develops in a very limited number of chronic alcoholics has definite biochemical features.It has been reported that oestrogens may precipitate or aggravate this variety of porphyria (Watson, 1960, 1964 ; Watson et al., 1962 ; Wilkinson, 1963 ; Hurley and English, 1963 ; Theologides et al., 1964) ; which, if true, might suggest a biochemical defect in alcoholic cutaneous hepatic porphyria similar to that present in acute intermittent porphyria-a disease known to be influenced adversely by female sex hormones.The cases of two patients who developed alcoholic cutaneous hepatic porphyria while receiving oestrogens for carcinoma of the prostate are described.We investigated the effect of oestrogen therapy on the levels of porphyrins in the blood and excreta of these two patients, and in six non-alcoholic patients with carcinoma of the prostate who had been taking stilboestrol in high dosage for a number of years.In neither group did the oestrogens affect the porphyrin levels. Case 1A retired labourer aged 81 presented with a six-month history of an eruption of tense bullae, circular ulcers, crusting, and scarring with patchy depigmentation on the areas exposed to light-bald pate, face, back of neck, and dorsa of hands and fingers.Cutaneous hepatic porphyria was diagnosed on clinical and histological grounds.The eruption had developed one month after prostatectomy for carcinoma of the prostate.(There were still no signs of metastases two years later.)The following drugs were given to him in hospital at the time of the prostatectomy: for anaesthesia, intravenous barbiturate (thiopentone), 400 mg., suxamethonium 50 mg., gall- amine 80 mg.; on one occasion only promethazine 50 mg.intra- muscularly, glutethimide 500 mg., Pamergan (atropine, pethidine, promethazine), and bendrofluazide 5 mg.; Pethilorphan (pethidine, levallorphan) 75 mg.four doses.He was discharged on caps.chlorotrianisene (Tace) 12 mg.b.d.There was no family history of a similar condition.He had been a healthy man who for about 30 years of his working life had drunk up to 14 pints (8 litres) of beer a day.On presentation he had no fever, no neurological or abdominal disturbance, and the liver and spleen were not felt.He had hyper- tension (210/180-120/100), radiological cardiomegaly, and a regular pulse rate of 60-90.Investigations.-Routine blood examination, urine examination, and chest x-ray picture were normal.Liver-function tests: brom- sulphalein test-less than 5% retention after 45 minutes; serum proteins-raised y-globulin level.Serum acid and alkaline phos- phatases were normal.Porphyrin results (Table I): porphyrins and porphyrin precursors were estimated over three periods-(1) before,(2) during, and (3) after stopping the oestrogens. Case 2A retired naval engineer aged 72 presented with an eruption of cutaneous hepatic porphyria that had developed eight months after