Autoimmune hemolytic anemia (AIHA) is a blood disorder in which your immune system attacks your own red blood cells. It can cause a wide range of symptoms, including fever, fatigue, a fast heart rate, shortness of breath, headaches, and nausea.

AIHA can also lead to skin changes. Knowing more about these symptoms may help you understand when you might be dealing with AIHA. If you notice these signs, talk to your doctor.

AIHA Skin Symptoms

Symptoms can vary from person to person, and some people with AIHA may not experience any skin changes. Additionally, there are two main types of AIHA, and each condition may be more likely or less likely to cause certain symptoms.

The most common type of AIHA is warm autoimmune hemolytic anemia. With this condition, the immune system’s antibodies attack red blood cells at a normal body temperature. Cold autoimmune hemolytic anemia, on the other hand, occurs when the immune system makes autoantibodies that become activated in colder temperatures.

Pale Skin

AIHA can cause the skin to appear paler or lighter. Paleness generally affects skin across the entire body. It is particularly noticeable on the skin of the lips and palms as well as the tongue, inside of the mouth, nails, and lining of the eyes. If these areas appear lighter than normal, you may be experiencing paleness related to AIHA.

In people with naturally darker skin, paleness may appear as a skin tone that is generally lighter or may look ashen, gray, or yellowish. Other areas such as the tissue around the eyes and in the mouth may appear lighter.

Pale skin is also a common symptom of other types of anemia (conditions that lead to a low red blood cell count). Red blood cells carry oxygen around the body using a protein called hemoglobin. People with anemia don’t have enough of these cells to keep a normal amount of blood flowing to the skin, which can then appear lighter.

Jaundice

Jaundice causes the skin to appear yellowish. This may be harder to notice in people with darker skin tones. However, jaundice also frequently makes the whites of the eyes and the mucous membranes, such as the inside the mouth, appear more yellow.

AIHA can cause jaundice by destroying too many red blood cells. Normally, your body continually makes new blood cells and gets rid of old ones. As old red blood cells break down, the body produces a substance called bilirubin. This chemical is usually processed by the liver. Some bilirubin is used to make bile, a fluid that helps digest food, and the rest is usually eliminated.

For a person has AIHA, the increased red blood cell destruction can lead to too much bilirubin within the body — so much that the liver may not be able to keep up. Because bilirubin is yellow, it makes some body tissues appear more yellow. It can also make urine look brown. If you have jaundice or dark urine, it may be a sign that your blood cells are breaking apart too quickly and your bilirubin levels are rising.

Changes in Skin Color

Cold AIHA, but not warm AIHA, can sometimes lead to skin discoloration when a person is exposed to cold temperatures. In particular, the skin of the fingers, toes, wrists, and ankles may begin to look different from normal.

Skin color changes may be the result of a condition called acrocyanosis, in which the skin looks dark purple or gray. Often, the periphery of the body — the ends of the limbs — is the first to get cold. When a person with cold AIHA spends time in cool temperatures, the immune cells begin attacking red blood cells, and the tissues in the hands and feet no longer get enough oxygen and begin turning blue.

A similar skin change may be caused by Raynaud’s phenomenon, a condition in which fingers appear a blotchy red or blue color. In one study, researchers looked at symptoms of 89 people with cold agglutinin disease (CAD), a subtype of cold AIHA. They found that 44 percent of people with CAD had acrocyanosis and 39 experienced Raynaud’s phenomenon or other related symptoms triggered by cold temperatures.

Necrosis and Gangrene

Some cases of severe acrocyanosis can turn into necrosis, a condition that occurs when tissue dies. Necrosis is painful and can make the skin become numb, develop blisters, or grow swollen.

Rarely, severe cases of Raynaud’s phenomenon can also lead to gangrene, in which large areas of tissue become necrotic and die due to too little blood flow.

Necrosis and gangrene are serious conditions. Seek emergency medical care if you think you might be experiencing either of these issues.

Sweating

AIHA can develop in two ways. Primary AIHA develops on its own, without any other related conditions. Secondary AIHA is associated with another illness or underlying disorder.

Two of the most common underlying conditions that can trigger AIHA are lupus and blood cancers such as chronic lymphocytic leukemia (CLL) or lymphoma. Lupus, an autoimmune disease, can cause sweating, fever, and feeling easily overheated. CLL and lymphoma often lead to a set of signs called B symptoms — drenching night sweats, fever, and unexplained weight loss.

People with secondary AIHA may experience unusual sweating due to their other related health conditions.

Flushing of the Face

Flushing or blushing isn’t a typical sign of AIHA directly. However, your skin may redden as a side effect of certain AIHA medications. For example, corticosteroids such as prednisone can cause facial flushing.

Talking to Your Doctor About Your Symptoms

If you notice any of these symptoms of AIHA, tell your health care provider. Some of them may be the result of health conditions other than AIHA. For example, pale skin frequently occurs with other types of anemia, and jaundice can be caused by liver problems, infections, or medications. Experiencing these symptoms does not necessarily mean you have AIHA. Additionally, AIHA is often accompanied by other, non-skin-specific symptoms.

There is a chance you could have AIHA if you have other disorders that could cause this condition, such as lupus, rheumatoid arthritis, ulcerative colitis, or certain thyroid problems. AIHA can also be caused by medications such as:

• Cephalosporins, a type of antibiotic
• Levodopa (sold under the brand names Sinemet, Parcopa, and Rytary)
• Penicillin or related drugs
• Quinidine
• Nonsteroidal anti-inflammatory drugs (NSAIDs)

Your doctor can use tests such as the Coombs test or a complete blood count to help diagnose AIHA or other potential health conditions. If you have already been diagnosed with AIHA and are experiencing ongoing skin issues, ask your doctor if other treatment options may help get your symptoms under control.

Connecting With Others

AIHA is a rare disease, but you’re not alone. On myAIHAteam, people with AIHA and their loved ones come together to ask questions, give advice, and share their stories with others who understand life with this condition.

Has your skin undergone changes as a result of AIHA? Share your experience in the comments below, or start a conversation by posting on your Activities page.