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If you’re living with paroxysmal nocturnal hemoglobinuria (PNH), you may notice changes in your skin. “The itch is so severe it hurts,” said one myPNHteam member.
PNH is a rare condition that is often recognized by one symptom: dark or cola-colored urine. This symptom is usually most noticeable in the morning. Although red blood cell breakdown (hemolysis) happens all the time, urine becomes more concentrated overnight, which presents as dark urine in the morning. However, PNH is a complex condition. People living with PNH can have many different symptoms, and sometimes this also includes skin-related symptoms.
In this article, we’ll cover four possible skin symptoms related to PNH.
People with PNH might notice an abnormally high number of bruises on their skin. This is because PNH can cause the skin to bruise easily. Even a light nudge or bump that normally wouldn’t produce a bruise may cause the skin to turn color, making it look like you were more seriously injured than you actually were.
Some people with PNH bruise easily because of thrombocytopenia, or a low number of platelets in their blood. Platelets are cell fragments in your blood responsible for blood clotting. They clump together to stop you from bleeding too much when you’re injured, even when an injury doesn’t break the skin.
Thrombocytopenia can happen in PNH because of the bone marrow failure or dysfunction caused by the condition. Bone marrow produces stem cells that become blood cells, including platelets, red blood cells, and white blood cells. When the bone marrow doesn’t function properly, it may produce fewer platelets than it usually would. Bone marrow failure in PNH can happen because of a related condition called aplastic anemia rather than from the PNH itself.
Thrombocytopenia can also happen when a part of the immune system called the complement system damages otherwise healthy platelets.
People with PNH who don’t have enough platelets bruise easily and tend to bleed a lot. This is because their blood doesn’t clot properly. Bruises form when blood doesn’t clot and instead gets trapped under the surface of the skin.
Bruises tend to go through a series of color changes as they heal.
For people with lighter skin tones, bruises often start out a blood-red color before darkening to a blue or purple tone. People with darker skin tones might have bruises that look dark purple, dark brown, or black at the beginning. Before the skin goes back to normal, it takes on a green or yellow tint. It can take weeks or months for a bruise to fade completely.
Many people with PNH have pale skin, which can make bruises stand out more. This can make even small bruises look darker or more noticeable than they usually would.
Pale skin in PNH is caused by anemia, which is a low red blood cell count. Similar to thrombocytopenia, anemia in PNH can happen when the immune system breaks down red blood cells or because of severe bone marrow dysfunction.
Many people with PNH have an associated condition called aplastic anemia, which is a bone marrow failure condition that can cause your bone marrow to make too few red blood cells. Aplastic anemia can lead to PNH, and some people with PNH can also develop aplastic anemia as a complication. While aplastic anemia doesn’t always cause symptoms, when it does, it can cause shortness of breath, along with skin symptoms like pale skin, rashes, and bruising.
Pale skin in people with PNH isn’t related to melanin (skin pigment). Instead, it’s caused by blood flow and red blood cell count.
Paleness in PNH affects the whole body, but it might be more noticeable in some areas than others. For example, you might notice that your skin is paler on your face or the inside of your mouth than on your arms and legs.
Paleness can also look different on different skin tones. In people with darker skin tones, paleness might be more noticeable in the linings of their eyes and mouth than in other areas.
People with PNH who also have anemia, including aplastic anemia, can also experience a skin symptom called anemia rash.
An anemia rash is most commonly seen with two types of anemia: aplastic anemia and iron-deficiency anemia. Aplastic anemia is closely linked with PNH. More than 10 percent of people with aplastic anemia eventually develop PNH. People with PNH are also at an increased risk of developing aplastic anemia later on. Because of this overlap, many people live with both conditions at the same time. As a result, people with PNH who also have aplastic anemia are more likely to experience an anemia rash.
When aplastic anemia causes anemia rash, it’s usually because of low platelet production from the bone marrow that leads to thrombocytopenia. When you have low platelet counts, the tiny blood vessels in your skin can bleed. This bleeding causes a red, dotted rash to appear.
Iron-deficiency anemia is also common in people with PNH because of chronic hemolysis (ongoing breakdown of red blood cells). Iron-deficiency anemia can sometimes contribute to anemia rash as well, but the connection between PNH and iron-deficiency anemia isn’t as well-studied. Iron-deficiency anemia might also worsen other types of anemia in PNH, including aplastic anemia.
Anemia rash causes petechiae, which are tiny red or purple dots just under your skin. These spots can appear in small patches or clusters anywhere on the body. In the rare cases when petechiae occur, they tend to show up most often on the fingers, toes, nose, and ears.
The rash may feel different depending on the type of anemia involved. When anemia rash is caused by iron-deficiency anemia, it often leads to itching. In contrast, anemia rash from aplastic anemia usually doesn’t itch or cause pain. Researchers aren’t sure why iron-deficiency anemia can cause itching, but they believe the itching might happen because iron-deficiency anemia thins the skin and makes it drier, which may lead to itching.
Jaundice, or yellowing of the skin, is another way PNH can affect a person’s skin tone.
Jaundice can develop in people with PNH for a couple of reasons. People with PNH might experience jaundice because of the breakdown of hemoglobin, the protein in red blood cells that gives them their red color. When hemoglobin breaks down, it produces a substance called bilirubin.
Typically, your liver processes this bilirubin and removes it from the body through your digestive system. In PNH, the high rate of hemolysis (the destruction of red blood cells) leads to high levels of bilirubin. When there is more bilirubin than your liver can handle, it builds up in the bloodstream. This buildup is what causes jaundice.
People with PNH can also get jaundice when blood clots cause blockages in the liver, preventing it from processing bilirubin properly. Between 15 percent and 30 percent of people with PNH experience thrombosis (blood clots that obstruct blood flow). Researchers aren’t sure why thrombosis is so common in PNH, but some believe it may be related to abnormal platelet development.
Another reason the liver might have trouble processing bilirubin is because of a rare PNH complication called Budd-Chiari syndrome. This condition causes inflammation and blood clots in the liver. In addition to jaundice, Budd-Chiari syndrome can cause other symptoms like abdominal pain and swelling.
Most people know of jaundice as yellow skin. However, the yellow color can also affect the whites of your eyes, the tongue, and mucous membranes such as the lining inside the mouth.
Ask your doctor if you notice any skin changes with PNH or worsening skin symptoms. New or worsening skin symptoms of PNH can be a sign that your condition is changing or that you’ve developed a complication like Budd-Chiari syndrome, which can be life-threatening without the proper treatment.
Your healthcare provider can determine the cause of your skin changes and recommend other treatment options if necessary.
On myPNHteam, people share their experiences with paroxysmal nocturnal hemoglobinuria, get advice, and find support from others who understand.
Which PNH skin symptoms have you experienced? Let others know in the comments below.
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