New Skin Changes? What Rashes, Bruising, or Wounds May Indicate in PI

If your immune system isn’t working the way it should, it can affect your entire body — including your skin. The skin is the largest organ in the body, and for people with primary immunodeficiency (PI), skin problems are common.

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Primary immunodeficiency is the general name for a group of hundreds of inherited conditions that weaken the immune system. Frequent or unusual skin issues can be early warning signs of PI. People living with PI also report a wide range of skin changes.

In this article, we’ll cover some of the most common skin changes associated with PI, what causes them, and what to do if you see any of these symptoms.

When Skin Problems Might Mean Primary Immunodeficiency

PI can be diagnosed in both children and adults. One of the key warning signs is infections that are frequent or severe or that keep coming back. People with PI may also get sick from germs that don’t usually make others sick. In addition, many people with PI develop autoimmune diseases, conditions that occur when the immune system mistakenly attacks the body’s own healthy cells.

Skin changes can be caused by infections or autoimmune activity, both of which are common in PI. Research suggests that around 48 percent to 55 percent of people with PI develop at least one skin-related symptom. In some cases, these issues are among the first signs of the condition. In one study, nearly 32 percent of people with PI reported that skin issues were their first noticeable symptom. These skin problems can include rashes, infections, or wounds that don’t heal easily.

Below are some of the possible skin changes that may occur with PI.

Common Skin Changes With Primary Immunodeficiency

The skin changes caused by PI can be divided into infections and noninfectious conditions. Infections are caused by viruses, bacteria, or fungi and may be spread through contact with other people or the environment. Noninfectious conditions are caused by the malfunctioning immune system and aren’t contagious.

Here are some of the common infections in PI.

Staphylococcus Aureus Infections

Often called staph, Staphylococcus aureus is a type of bacteria that lives on the skin. It causes millions of skin infections every year, but they’re usually mild and go away with antibiotic treatment. Staphylococcus bacterial infections are the most common skin issue reported in a few different PI conditions. Having repeated or severe staph infections might be a sign of PI.

A typical staph skin infection causes discolored, pus-filled, painful sores on your skin. However, staph infections seen in people with some types of PI can be much more serious. In these cases, staph bacteria can infect organs or enter the blood. The infection can move quickly and be life-threatening.

Warning signs of a serious staph infection include:

• Flu-like symptoms, including fever, chills, fatigue, and cough
• Pain, swelling, warmth, and discoloration around the affected area

If you have any of these signs or symptoms, get medical help as soon as possible.

Viral Skin Infections

Severe, hard-to-treat skin infections with a few different viruses are described for some types of PI. There are vaccines for many of these viruses, but people with PI may not respond well to them or may not be able to receive them safely. Here are some of the more common viral skin infections linked to PI.

Human Papillomavirus

There are hundreds of strains of human papillomavirus (HPV). Some of them, especially the low- or medium-risk strains, may cause warts on the skin or genitals. Some high-risk strains can contribute to the development of squamous cell carcinoma, particularly in mucosal areas. People with some PI conditions are at higher risk of having severe HPV infection, which may involve warts covering large areas of the body. They may also have a higher risk of skin cancer caused by HPV.

Herpes Simplex Virus

Herpes simplex virus causes painful, fluid-filled ulcers on the skin or in parts of the skin called mucous membranes. These are moist areas in and around the mouth, nose, and genitals. People with some forms of PI are more prone to severe herpes infections. The ulcers may be larger and take weeks or months to heal. The infection can also progress into the lungs or throat and cause symptoms like a sore throat and cough.

Varicella Zoster

The varicella zoster virus causes chickenpox, an itchy spotted skin rash, during a first infection. The virus stays in your body and can be reactivated to cause shingles. People with mutations (changes) affecting their T cells or natural killer (NK) cells — two types of white blood cells — are at higher risk of severe infection with varicella zoster.

Molluscum Contagiosum

Molluscum contagiosum causes white, pink, or skin-colored bumps the size of a pencil eraser anywhere on the body. In people with PI, the bumps may cover a large part of the skin. The bumps might also be unusually large or look like skin cancer.

Fungal Skin Infections

Frequent or hard-to-treat fungal infections can be an early warning sign of PI. One common fungus, Candida, can cause recurring infections in both children and adults with PI. When Candida affects the mouth or throat, it’s called thrush. But this fungus can also infect the skin, scalp, and nail beds. It most often occurs in moist, damp, and creased areas like the armpits and groin.

Symptoms of Candida infection may include:

• An intensely itchy, spreading rash
• Discolored rashes in skin folds and creases
• Folliculitis (infected hair follicles) — small, red or white bumps that look like pimples
• Changes in nail appearance, such as misshapen, discolored, or brittle nails

Noninfectious skin changes that can happen with PI include the following.

Vasculitis

Vasculitis is an autoimmune condition in which blood vessels become inflamed. This inflammation makes it harder for blood to flow. When too little blood gets to your organs and tissues, it can cause many different symptoms.

Some of these include:

• Rashes, bumps, or areas of purple spots or other discoloration on the skin
• Unexplained weight loss
• Joint pain
• Fever
• Numbness or tingling in hands and feet

Doctors are increasingly recognizing that vasculitis can be a potential complication of PI. In a study of people with PI in the United States, 1.6 percent were also diagnosed with vasculitis.

Eczema

Eczema is an inflammatory condition that causes itchy, discolored, scaly patches on the skin. The most common form of eczema is atopic dermatitis. Scratching eczema patches can break the skin and lead to secondary infections, especially in people living with PI.

Eczema is the most common autoimmune skin condition in PI. Research shows it affects between 13 percent and 22 percent of children diagnosed with PI.

Eczema in PI often looks similar to eczema in the general population, but it may be more widespread, more persistent, and more prone to infections. If your eczema is severe or difficult to manage, talk with your doctor — it could be a clue to an underlying immune issue.

Skin Changes and Primary Immunodeficiency: What To Do Next

If you or someone you care for is noticing new or ongoing skin changes, talk with a healthcare provider. Symptoms like persistent rashes, recurring infections, or slow-healing sores can be early signs of a problem with the immune system. This is especially true if skin symptoms appear alongside other health concerns, such as autoimmune conditions.

Your doctor may recommend blood tests or a skin biopsy (a small sample of skin) to help determine whether the issue is related to PI or another cause, such as secondary immunodeficiency (which develops later in life from factors like medications or infections).

If you’ve already been diagnosed with PI, let your healthcare team know about any new or worsening skin changes. Early testing and treatment may help reduce discomfort, lower the risk of complications, and improve your daily quality of life.

Find Your Team

On myPIteam, people come together to learn more about life with primary immunodeficiency disorders.

Have you or a loved one been diagnosed with a primary immunodeficiency disorder? Have you sought medical advice or medical treatment for any of these skin conditions? Share your tips and experiences in a comment below.