PYROLURIA: If You Have It, You Need To Know it!

Pyroluria: The Most Common Unknown Disorder


Pyroluria Cover

Pyroluria:  The Unknown Disorder

Pyroluria, also called Malvaria, is a unique metabolic condition that is very rarely recognized in both the medical and natural health world.   Some researchers in the orthomolecular medicine and orthomolecular psychiatry believe that up to 10% of the population has this metabolic condition (1).  It is considered by many in the functional medicine world the most common unknown disorder.  Pyrolurics need a specific diet, lifestyle and supplementation program to get well.

Pyroluria is a genetic condition that is typically related to familial alcoholism and/or environmental toxicity.  If an individual has a family history of alcoholism they may very well have this genetic mutation.  It can be induced with childhood trauma or a chronic infection early in life.  The onset usually begins in the late teens and is often triggered by a traumatic life event.

The Symptoms Associated with Pyroluria:

It is estimated that as many as 50% of those with autism, 40% of alcoholics, 70% of schizophrenics, 70% of depressed individuals and 30% of people with ADHD have pyroluria (23).  When this goes undiagnosed and untreated it becomes very challenging for these individuals to ever get well despite the use of holistic therapies.  Most will struggle with their health throughout their life and never find any long-term answers.

The symptoms of pyroluria include chronic anxiety, poor stress tolerance, digestive issues, poor immunity, joint pain, acne or eczema, mood swings and poor short term memory.  These individuals often have difficulty digesting and absorbing protein and they are easily wrecked by increasing stress (456).

Diagnosing Pyroluria:

Pyroluria can be diagnosed through a specific Kryptopyrroles urine test.  Normal amounts of kryptophyrroles in the urine should be less than 20 ug/dl.  Numbers greater than 20 ug/dl would be a positive test for the diagnosis of pyroluria (7).  Many clinicians have found that this number should be less than 15 ug/dl.

The lab that I use for this test measures kryptopyrroles in mcg/dl and the number should be less than 9 mcg/dl.  Anything between 10-15 is considered mild pyroluria, 15-19 is moderate and anything above 20 mcg/dl, we consider a severe case.

Other warning signs you may have this disorder include an inability to remember dreams, hairloss, light, thin pale skin that is prone to stretch marks, white spots on the fingernails and poor tooth enamel (8).


What is Pyroluria:

There are several waste products that are produced when the body makes hemoglobin for the red blood cells.  These waste products are called kryptopyrroles which are technically called hydroxyhemoppyrrolin-2-one (HPL) which are typically excreted by our body.  Individuals with pyroluria are unable to clear the HPL effectively and they build up in the system (910).

The HPL binds strongly to zinc, biotin and vitamin B6 which are critical nutrients for cellular metabolism.   Over a period of time, the body becomes very deficient in these critical nutrients and symptoms arise.  These people will often go years suffering the effects of this disorder despite a clean diet, supplementation and holistic therapies.


Nutrition & Supplementation:

Pyrolurics have a greater need for omega-6 fatty acids than most in our society (1112).  They need both arachidonic acid (AA) and Gamma linolenic acid (GLA).  A diet rich in pastured eggs, pasture-fed butter, grass-fed beef and wild game and organ meats supplies the body with significant amounts of essential fats.  These foods are also rich in zinc.

GLA is found in borage oil, evening primrose oil, black current seed oil and hemp oil.  This is typically supplemented with one of these sources as it is hard to get enough in our diet.

These individuals need to supplement with zinc and co-enzymated form of vitamin B6 called P-5-P (1314).  A general methyl donating supplement with P-5-P and other methyl donors such as trimethylglycine, methyl-folate, methyl-B12 and riboflavin 5’phosphate sodium is quite effect with this process.

Vitamin B6 is found in seafood, meat and green leafy veggies among other things.  However, the number one way we get B6 into our system is through fiber that our gut microbes break down and produce B6 as a byproduct of their metabolism of the fibers.   Probiotics and fermented foods are also necessary for these individuals to reduce inflammatory gut related stress and heal appropriately.

Lifestyle & Supplements Get Results:

With the right diet, supplementation and stress reduction most pyrolurics see results very quickly.  More severe cases usually experience slow progressive results.  Very stubborn cases are typically having issues with heavy metals or mold toxins.

They will need to remain on this lifestyle with moderate supplementation for the rest of their life or risk symptoms coming back.  Typically, large doses of supplementation is used in the beginning and the individual tapers down to smaller doses as they feel better and have a return to nutrient sufficient status.

Clinically, I use high doses of zinc, methylation support, mitochondrial support (CoQ10, Lipoic Acid, N-Acetyl Cysteine), Probiotics and GLA.


Testing for Zinc and B6 Status:

The easiest way to test for zinc sufficiency is through a zinc tally test.  This is a test done by tasting a liquid zinc supplement.  With zinc sufficiency the liquid tastes awful but those who are deficient in zinc will not taste the liquid supplement.  This is a positive flag for a zinc deficiency.  Blood work could also be done to verify but most experts like the zinc tally test as it is easy and inexpensive.

The subjective test for B6 sufficiency is the return of regular dreaming that the individual can remember.  They don’t have to remember the entire dream but bits and pieces is a sign of sufficiency.  When they are deficient they will typically be unable to remember anything about their dreams (15).

Additional Tips to Know:

Pyrolurics also need to be sure to eat very clean and avoid phytate containing foods such as grains, nuts, legumes and soy.  Phytic acids in these foods bind to major minerals like zinc and render them useless in our body (16).  Soaking and sprouting legumes and nuts reduces the phytate count.

Due to zinc deficient states they are more susceptible to heavy metal toxicity from mercury, cadmium and copper.  When the individual is unable to develop a zinc sufficient state despite very large supplemental usage it is most likely related to heavy metal toxicity.  Special testingcan be done for heavy metal levels.

Chronic stress, alcohol consumption, sugar consumption, smoking and pharmaceutical drugs will all further aggravate the symptoms and severity of pyroluria.


Pyroluria Questionnaire:

The following includes the most common symptoms associated with the condition Pyroluria.  If you answer “yes” to 15 or more of these then further testing may be worthwhile:

1. Little or no dream recall

2. White spots on finger nails

3. Poor morning appetite +/- tendency to skip breakfast

4. Morning nausea

5. Pale skin +/- poor tanning +/- burn easy in sun

6. Sensitivity to bright light

7. Hypersensitive to loud noises

8. Reading difficulties (e.g. dyslexia)

9. Poor ability to cope with stress

10.  Mood swings or temper outbursts

11.  Histrionic (dramatic) tendency

12.  Argumentative/enjoy argument

13.  New situations or changes in routine (i.e., traveling) particularly stressful

14.  Much higher capability and alertness in the evening, compared to mornings

15.  Poor short term memory

16.  Abnormal body fat distribution

17.  Belong to an all-girl family with look-alike sisters

18.  Dry skin

19.  Anxiousness

20.  Reaching puberty later than normal

21.  Difficulty digesting, a dislike of protein or a history of vegetarianism

22.  Tendency toward being a loner and/or avoiding larger groups of people

23.  Stretch marks on skin

24.  Poor sense of smell or taste

25.  Feel very uncomfortable with strangers

26.  Frequently experience fatigue

27.  A tendency to overreact to tranquilizers, barbiturates, alcohol or other drugs (in other words, a little produces a powerful response)

28.  A tendency toward anemia

29.  History of mental illness or alcoholism in family

30.  Easily upset by criticism

31.  Sweet smell (fruity odor) to breath or sweat when ill or stressed

32.  Prone to acne, eczema or psoriasis

33.  A tendency toward feeling anxious, fearful and carrying lifelong inner tension

34.  Difficulty recalling past events or people

35.  Bouts of depression or nervous exhaustion

36.  Prone to frequent colds or infections

I have a specific blood test that lets me know if someone is positive for the Pyroluric condition and I have a specialized nutrition and supplement protocol to help these individuals regain their health.

Sources For This Article Include:

1. Nutritional Healing – Pyroluria

2. HOFFER A, OSMOND H. MALVARIA: A NEW PSYCHIATRIC DISEASE. Acta Psychiatr Scand. 1963;39:335-66. PMID: 14078702

3. Gebel L. [Occurrence of the mauve factor in schizophrenia]. Psychiatr Pol. 1973 Mar-Apr;7(2):153-9. Polish. PMID: 4710987

4. HOFFER A, MAHON M. The presence of unidentified substances in the urine of psychiatric patients. J Neuropsychiatr. 1961 Aug;2:331-62. PMID: 13714995

5. HOFFER A. The presence of malvaria in some mentally retarded children. Am J Ment Defic. 1963 Mar;67:730-2. PMID: 13963913

6. Ellman GL, Jones RT, Rychert RC. Mauve spot and schizophrenia. Am J Psychiatry. 1968 Dec;125(6):849-51. PMID: 5698458

7. A Rapid Screening Test for Pyroluria; Useful in Distinguishing a Schizophrenic Subpopulation

8. Hoffer A. Malvaria and the law. Psychosomatics. 1966 Sep-Oct;7(5):303-10. PMID: 5912644

9. Walker JL. Neurological and behavioral toxicity of kryptopyrrole in the rat. Pharmacol Biochem Behav. 1975 Mar-Apr;3(2):243-50. PMID: 1096175

10. Krischer K, Pfeiffer CC. Biochemical relationship between kryptopyrrole (mauve factor and trans-3-methyl-2-hexenoic acid (schizophrenia odor). Res Commun Chem Pathol Pharmacol. 1973 Jan;5(1):9-15. PMID: 4686114

11. Heleniak EP, Lamola SW. A new prostaglandin disturbance syndrome in schizophrenia: delta-6-pyroluria. Med Hypotheses. 1986 Apr;19(4):333-8. PMID: 3520252

12. Horrobin DF, Huang YS. Schizophrenia: the role of abnormal essential fatty acid and prostaglandin metabolism. Med Hypotheses. 1983 Mar;10(3):329-36. PMID: 6348496

13. Treatment of Pyroluric Schizophrenia (Malvaria) With Large Doses of Pyridoxine and a Dietary Supplement of Zinc

14. Zinc and Manganese in the Schizophrenias

15. Ebben M, Lequerica A, Spielman A. Effects of pyridoxine on dreaming: a preliminary study. Percept Mot Skills. 2002 Feb;94(1):135-40. PMID: 11883552

16. Torre M, Rodriguez AR, Saura-Calixto F. Effects of dietary fiber and phytic acid on mineral availability. Crit Rev Food Sci Nutr. 1991;30(1):1-22. PMID: 1657026