When the body breaks down hemoglobin in red blood cells, it creates byproducts—kryptopyrroles (KPU), and hydroxyhemopyrrolin-2-one (HPL). Most people excrete these in the urine without a problem—but in some cases, they accumulate. This is a problem, because pyrroles and HPL bind to Vitamin B6, and to zinc, causing deficiencies.
The condition is variously called pyroluria, kryptopyroluria, or the Mauve Factor.
Symptoms of Pyroluria
Symptoms of pyroluria correspond to symptoms of deficiency in Vitamin B6 and zinc.
Zinc is one of those ubiquitous nutrients that acts as a cofactor for many different enzymatic reactions in the body. Zinc deficiency causes depression or changes in brain chemistry, affecting GABA levels, and also leading to a corresponding rise in copper (since copper and zinc are like a seesaw: as one goes down, the other goes up, and vice versa). High copper can lead to high levels of catecholamines (dopamine, epinephrine and norepinephrine), associated with mood disorders like schizophrenia, anxiety, OCD, hyperactivity, Oppositional Defiant Disorder, bipolar disorder, or attention issues.
Zinc is also a critical nutrient to help maintain the health of the gut—so low levels can lead to low production of pancreatic enzymes, bloating, nausea, and leaky gut syndrome.
Zinc deficiency also presents with white spots on nails, hair loss, low appetite, lack of taste, amenorrhea (no periods), poor essential fatty acid metabolism, increased sensitivity to pain, low melatonin (so sleep issues), stretch marks, poor wound healing, growth retardation, low sex hormones, intense reactions to lights and sounds, and decreased stress tolerance.
Low Vitamin B6 can exacerbate the brain chemistry problem, since it is a necessary cofactor for the body’s production of a number of neurotransmitters, including GABA, serotonin, melatonin, dopamine, norepinephrine, and epinephrine. Lower levels of B6 will lead first to lower serotonin and GABA, though. This is probably why low B6 is itself associated with insomnia and depression, as well as menstrual irregularities.
It’s also necessary to produce glutathione, arguably the most powerful antioxidant in the body. It’s also required for one of the liver’s six main pathways of Phase 2 Detoxification, and it offers immune support and cytokine balancing in chronic illness.
B6 deficiency also causes poor dream recall, dermatitis, cheilosis (cuts on the sides of the lips), and peripheral neuropathy.
Causes of Pyroluria
Pyroluria can be genetic, and in cases where some of the above symptoms show up in kids, this may be the cause. But there are other possible causes as well: leaky gut syndrome, and stress.
According to this study, heightened pyrrole and HPL excretion correlates with stress of all kinds, including emotional stress, physical stress such as an illness or infection, and oxidative stress (i.e. low antioxidant status). Correspondingly, prednisone (a much more powerful version of the body’s stress hormone cortisol) increases HPL excretion.
Stress can also trigger leaky gut syndrome, since cortisol thins the gut lining (cortisol thins tissue in general). And sure enough, this study shows that urine levels of HPL correlate with intestinal permeability. Antibiotics also lower levels of HPL in urine, suggesting that dysbiosis may play a role in pyroluria.
Testing and Treatment for Pyroluria
Testing involves looking for both KPU and HPL. If positive, treatment of course requires supplementation with Vitamin B6 and zinc, but at doses very high doses. It’s possible to overdose on both B6 and zinc, so I’d definitely test before treating.
Since levels of many vitamins and minerals affect others, high doses of zinc and B6 (even when needed) may deplete other minerals—so it may be necessary to supplement with those too, particularly manganese and magnesium. Niacinamide (Vitamin B3), Vitamin C, and Pantothenic Acid (Vitamin B5) can also help speed recovery.
