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Understanding Pyroluria

Understanding Pyroluria

What’s in a name?

The mauve factor (Malvaria), also known as hydroxyhemopyrrolin-2-one (HPL), kryptopyrrole and urinary pyrrole, was first reported in 1961. Pyroluria is a blood disorder that affects the synthesis and metabolism of the oxygen carrying component of red blood cells, haemoglobin.  All cells release and produce wastes as part of normal function.  The by-product of haemoglobin is the metabolite called hydroxyhemopyrrolin-2-one (HPL) also known as Pyrrole. In people with pyroluria, these by-products or HPL accumulate in the body and if they are not cleared they can bind to other essential nutrients causing deficiencies. HPL binds to vitamin B6 (pyridoxine), zinc reducing the levels of these vitamins in the body.

Reduced B6, zinc and deficiencies of other B-vitamins, manganese, arachidonic acid and chromium can occur. Potential increased copper levels and less efficient oxygenation of the body can contribute to a vast array of signs and symptoms.  B6 and zinc are needed for the proper metabolism of dietary omega-6 fatty acids and the conversion of this to GLA (also needs B3), into anti-inflammatory prostaglandins (they are produced at many target sites throughout the body to prevent inflammation). Deficiencies in B6 and zinc may contribute to immune dysfunction, anxiety, depression, heavy metal toxicity, mineral imbalances amongst others.

Signs and Symptoms

White spots on finger nails Insomnia
Little or no dream recall Stress intolerance
Pale skin (face), poor tanning, easily burnt Sensitive to bright light, loud noise & odour
Poor sense of taste or smell Anxiousness, fearful, irritable
Digestive problems especially protein Dry skin
Explosive temper and mood swings Loner tendencies or dislike of large groups
Fatigue Body fat (abdominal) distribution
Tendency to depression or nervous exhaustion Frequent colds and infections
Tendency toward anaemia Difficulty recalling past events/people
ADHD symptoms Poor short term memory
Triggers:  Stress, Travelling, Trauma Prone to acne, psoriasis, eczema
Family history of mental illness or alcoholism Hypoglycaemia
Delayed puberty, stunted growth Low morning appetite/morning nausea
Reading difficulties Sensitivity to alcohol, tranquilisers, drugs
Increased alertness in the evening vs mornings Stretch marks on skin
Unpleasant body odour/fruity breath Easily upset by criticism
Cold hands or feet Constipation
Migraines Pessimism
Social Withdrawal Irregular periods

Causes of pyroluria have been attributed to genetic predisposition, early childhood trauma or chronic infections. It was found that high urinary levels of Mauve (pyrroles) were present in a proportion (up to 80%) of schizophrenic patients when studies were first carried out. On remission, the level of Mauve in these individuals reduced to normal amounts. Subsequent studies have shown that high-Mauve is not specific to schizophrenia, but is also elevated in alcoholism, autism, criminal behaviour, depression, Down syndrome and many other mental health disorders. As many as 10-15% of all people suffer from pyroluria. It is a lifelong condition, and symptoms tend to worsen with age and stress.healthy-food


Allow your naturopath to calculate using your levels of serum copper, zinc and ceruloplasmin to work out the unbound or free copper that is causing damage. there is another way listed below using urine however I recommend using your blood pathology from your doctor or organised through Karen as the expense is a lot less. It also determines the copper:zinc ratio with the unbound copper amount.

Pyrroles are sensitive to light and you will need to use a specialised facility for testing if through urine.  Before testing avoid all nutritional supplements (especially those containing zinc, vitamin B6 and biotin) for 5-7 days.


This usually involves the use of vitamin B6 and zinc, possibly manganese, biotin and arachidonic acid. As each individual is different, dosages may need to be adjusted to achieve optimal levels. Those with pyrroles can also benefit from taking evening primrose oil capsules.

Pyroluria is a condition that requires lifelong monitoring, however lower doses and the use of “food as medicine” or lifestyle interventions may be trialed once nutrient levels are replete and the person is stable.

Pfeiffer, who was one of the initial investigators of Mauve, treated more than one thousand high-Mauve patients with vitamin B6 and zinc with positive results. Symptom improvement with these supplements can be experienced in patients in as little as two days and Mauve levels have been observed to decrease by as much as 50% after 1 month of treatment. However, discontinuation of the supplement regime may can result in deterioration of the patient in as little as 48 hours.  Severe cases may take longer (3-6 months) to treat. Nutrient dosages are prescribed dependent on your presenting case and severity of your symptoms.

Many neurotransmitters such as serotonin, dopamine and gamma aminobutyric acid (GABA) require vitamin B6 and/or zinc for their production. It is therefore not surprising that individuals with high-Mauve levels, and therefore vitamins B6 and zinc deficiencies, may have neurological imbalances.

Stress management

Stress and prolonged periods of stress can increase the severity of symptoms. It is therefore important to adopt on-going techniques such as yoga, meditation, mindfulness or counselling to manage and low stressful situations.  Alcohol should be avoided as it results in lowered amounts of B6.  Several B-vitamins are needed to assist people with pyrroles.

Eating wholefoods and foods that are low GI, adequate protein, fibre, probiotic supplementation, and vegetables in the diet is recommended.  High GI foods and refined carbohydrates, are best keep to a minimum, as microflora balance and production of neurotransmitters that reduce anxiety are important.  Staying hydrated is also important.  Copper is also essential for health so you don’t want to remove all of it. Read my blog here on coppers affects in health.

Hormonal management involvement

Nutrient deficiencies, increase copper levels and low fibre may influence oestrogen and thyroid hormone levels and treatment may be required to correct any hormonal imbalances.

Feel you need to be tested?  Treatment plans can be made with Karen on 0400836254. Karen is a fully qualified Naturopath, nutritionist & herbalist with full membership through the NHAA Read more about Karen here

More information

The New Black

Mauve Factor

Gaining Health Naturally Naturopathy Brisbane, Sunshine Coast Qld

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