SYNOPSIS In intermittent acute porphyria there are no specific alterations in the ultrastructure of the liver. In contrast, the constant characteristic of porphyria cutanea tarda is siderosis of the hepatocytes and Kupffer cells. Therefore, between the two types of hepatic porphyria there are no similar ultrastructural lesions. It is still doubtfulwhether the presence of hepatosiderosis in porp...

M ANY CASES of porphyria cutanea tarda have been reported since the term was introduced by Waldenstr#{246}m in 19371 to describe a type of porphyria occuring usually in adult patients and characterized by the presence of vesicles and bullae over areas exposed to the sun. Porphyria cutanea tarda may be on a genetic basis as seen frequently in the white population of South Africa,24 or it may be ...

M ANY CASES of porphyria cutanea tarda have been reported since the term was introduced by Waldenstr#{246}m in 19371 to describe a type of porphyria occuring usually in adult patients and characterized by the presence of vesicles and bullae over areas exposed to the sun. Porphyria cutanea tarda may be on a genetic basis as seen frequently in the white population of South Africa,24 or it may be ...

Porphyria cutanea tarda is a metabolic disorder in the haem biosynthetic pathway. It includes a heterogeneous group of conditions, which may be inherited or, more commonly, acquired. Although porphyria cutanea tarda presents with cutaneous lesions only, it is often associated with systemic disease. A 64-year-old Chinese patient, who developed sporadic porphyria cutanea tarda 1 year after the di...

M ANY CASES of porphyria cutanea tarda have been reported since the term was introduced by Waldenstr#{246}m in 19371 to describe a type of porphyria occuring usually in adult patients and characterized by the presence of vesicles and bullae over areas exposed to the sun. Porphyria cutanea tarda may be on a genetic basis as seen frequently in the white population of South Africa,24 or it may be ...

A woman aged 54 was studied because of a severe acute porphyric (neurologic) relapse with clinical and chemical findings characteristic of porphyria variegata. During a family survey, her brother, aged 59, was found to have chemical abnormalities typical of porphyria cutanea tarda, without suggestion of neurologic manifestations. He had mild skin changes compatible with either of these forms of...

A 42 year old heterosexual male with symptomatic human immunodeficiency virus infection presented with a 2-week history of tense blistering skin lesions following azidothymidine therapy. Urinary porphyrin excretion confirmed the diagnosis of porphyria cutanea tarda. The blisters resolved following the withdrawal of the drug but recurred when rechallenged. Three other cases of porphyria cutanea ...

A female patient aged 73 presented with a history of general malaise and hyperpigmentation. Iron studies in the patient and immediate family members indicated that the proband was homozygous for haemochromatosis, but subsequent investigations revealed that porphyria cutanea tarda was responsible for her signs and symptoms. Venesection of four units of blood brought her symptoms under control. T...

In intermittent acute porphyria there are no specific alterations in the ultrastructure of the liver. In contrast, the constant characteristic of porphyria cutanea tarda is siderosis of the hepatocytes and Kupffer cells. Therefore, between the two types of hepatic porphyria there are no similar ultrastructural lesions. It is still doubtful whether the presence of hepatosiderosis in porphyria cu...

BACKGROUND Porphyria is an umbrella term for a group of largely hereditary diseases that are due to defective haem synthesis. The diseases have a varied and partly overlapping range of symptoms and presentations. The commonest forms of porphyria are porphyria cutanea tarda, acute intermittent porphyria and erythropoietic protoporphyria. The purpose of this study is to provide an overview of the...