The porphyrias

Porphyrias are caused by deficiencies of one of several enzymes responsible for the production of haem. The form of porphyria is determined by which enzyme is deficient. Symptoms of porphyrias are caused when intermediates of the haem-synthesis process accumulate in abnormal and toxic amounts.(58) The most common forms of porphyria are acute intermittent porphyria, porphyria cutanea tarda, and erythropoietic porphyria.

Symptoms and signs of acute intermittent porphyria include abdominal pain, vomiting, constipation, muscle weakness, hypertension, tachycardia, peripheral neuropathy, and mental status changes.(58) The most prominent mental status changes are anxiety, depression, and suspiciousness. Precipitating factors for attacks include alcohol, fasting, and a number of medications. Treatment is symptomatic and preventive; no primary treatment exists and patients should avoid alcohol and excessive dieting. Narcotic analgesics are safe during attacks.

Porphyria cutanea tarda is precipitated by alcohol and sex hormones, particularly oestrogen. Symptoms include skin vesicles and bullae, hyperpigmentation, and skin fragility when exposed to significant light. Prevention of attacks is the focal point of management. Patients should avoid excessive exposure to sunlight, alcohol, and oestrogen. Erythropoietic porphyria also manifests skin-related signs and symptoms, including burning, oedema, itching, and erythema. Limitation of sun exposure is the primary form of prevention.

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Peripheral Neuropathy Natural Treatment Options

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