ABSTRACT
The porphyrias are a group of disorders of metabolism of the haem molecule. Acute, intermittent porphyria has no skin manifestations. Porphyria cutanea tarda is a so-called hepatic porphyria. There is a genetic component to the disorder, although it has not been completely characterized. It is much more common in those with alcoholic liver disease, but has also been seen in patients with liver tumours and those with hexachlorbenzene poisoning. In the early stages of the disease, blistering and fragility of the skin metabolic disorders and reticulohistiocytic proliferative disorders on the face and backs of the hands are noted. Erythropoietic protoporphyria is a very rare, autosomal dominant disorder in which excess protoporphyrins are produced. Amyloidosis is the term used for a group of disorders in which an abnormal protein is deposited in tissues. Xanthomata are deposits of lipid in histiocytes in skin and may be associated with normal levels of lipids in the blood or with elevated levels of serum lipids.
