Intermittent (hepatic) porphyria

ABSTRACT

A fifty-year old Curacaoan negro woman presented the clinical features of intermittent (hepatic) porphyria. Bullous skin lesions on hand and legs were concomittant. Family examination was negative. Neurological and psychiatric symptoms as well as severe abdominal distress marked the first attack. Biopsies of nerve, skin and liver were performed. A biochemical follow-up is presented. A chromatografic pattern of the urinary porphyrins showed that this patient belong to the so-called unclassified group of porphyrins. Clinically she was of the mixed type of porphytis (variegate porphyria) (AU)