Medical Matters - What your skin may be trying to tell you.

Your skin is the largest organ in your body, and it has a lot to say about your health. Therefore, it is important to pay close attention to it, especially if you have a primary immune deficiency.

By Rachel Maier, MS

Your Skin communicates nonverbal messages all the time. A glowing tan reveals you spent time in the sun; goosebumps say you’re cold, frightened or excited; stretch marks indicate a recent pregnancy or weight loss. Your skin also gives you clues that distress or disease is happening somewhere else in your body. For example, dry skin can indicate dehydration or diabetes; flushed skin can signal you are overheated or have taken too much of a certain medication; and mottled skin can indicate poor circulation or melasma. When something about your skin changes, pay attention. Your skin may be saying something simple, but it could be telling you something much more important.

Skin Changes vs. Skin Diseases

Skin changes all the time. Some changes are normal, natural and do not pose a danger to your health (although sometimes, they may feel uncomfortable). For example, acne usually breaks out during adolescence; freckles may appear after spending extended amounts of time outside; and aging skin usually develops fine lines and wrinkles. These messages signal changes are happening in your body, but they usually are not a cause for concern. However, some changes in your skin are not normal. Things like hives; atopic dermatitis (eczema); new growths or moles; redness, swelling or pain; changes in color or texture; or pus-filled blisters may indicate an underlying condition that needs medical attention. If your skin develops patches of discolored skin; open sores, lesions or ulcers; itchy, painful rashes; red, white or pus-filled bumps; unusually scaly or rough skin; or moles that change shape, color or size, make an appointment with your doctor. Medical Matters Your doctor may order one or more lab tests, including blood tests, biopsies and skin cultures to help identify the cause. Blood tests look for autoantibodies, bacteria or viruses; biopsies use a needle, punch or cutting sheath to remove a small piece of skin that is analyzed for skin disorders, infections or cancers; and skin cultures use a swab, needle or syringe to collect a sample of skin to look for germs that may be causing an infection.

Noninfectious Skin Conditions

Noninfectious skin conditions are common and usually harmless. They often can be tolerated with comfort care at home, but some may require medical intervention. However, many noninfectious skin conditions can point to an underlying issue, so it is a good idea to talk to your medical provider about them. Here is a snapshot of the most common noninfectious skin conditions:

• Eczema. This chronic condition causes inflammation, redness and irritation of the skin. Also called atopic dermatitis, symptoms include red, dry patches of skin; rashes that ooze, weep or bleed when scratched; or thickening and hardening of the skin. During childhood, Reprinted with permission Ig Living. What your skin may be trying to tell you. Castaneous Cues: a red, thickened rash that may ooze or bleed is most common on the elbows, knees, neck and ankles. In teens and adults, the most common symptom is a red to dark brown scaly rash, which may bleed or crust when scratched, that appears on the hands, neck, elbows and knees, skin around the eyes and ankles and feet. Eczema is not contagious; an interplay of genes, the immune system and the environment produce this often painful condition. Those with asthma, allergies or hay fever are prone to eczema. Bacterial skin infections can develop in those with eczema, usually from scratching and infecting an irritated area.

• Psoriasis. Psoriasis is a chronic, often painful skin disease characterized by rashes that appear as dandruff-like plaques or scaly patches on the skin, usually on the knees, elbows, trunk and scalp. It is an autoimmune disease without a known cure, and people who have it are at risk for developing skin infections.

• Granulomas. Granulomas are small clusters of immune cells that form when the immune system protects itself from something harmful or foreign such as bacteria, a fungus, a virus or even splinters or sutures. Granulomas are more common in people with chronic infections or inflammation, but anyone can get them. These masses are not usually dangerous on their own, but instead may point to a chronic condition such as chronic granulomatous disease (CGD), sarcoidosis or an infection. Granulomas can form anywhere on the body, but they usually appear on the skin as raised, red ring-shaped lesions.

• Telangiectasias. This is a big word for a simple condition: broken capillaries. Commonly called “spider veins,” telangiectasias are damaged capillaries that are visible just beneath the skin. They appear as red or purple web-like clusters that resemble tree branches or spider webs. They can form anywhere, but they typically develop in the legs or face. Telangiectasias are common in autoimmune conditions such as scleroderma, dermatomyositis and lupus, and links between telangiectasias and primary immune deficiencies have been found.

• Hives. Also called uticaria, hives are raised, red or skin-colored itchy bumps that can appear after exposure to allergens (food, medicine, latex, pet dander, pollen), insect stings or bites, infections or even physical stimuli (pressure, cold/heat, exercise, sun exposure). Hives can appear anywhere and often change shape, move around and disappear and reappear over short periods of time. They can be either acute (short-lived) or chronic (long-term). Antihistamines usually resolve the symptoms of hives by blocking the effect of histamine (the chemical released by the immune system in response to a foreign protein). Hives are not dangerous on their own, but when they are accompanied by swelling of the throat or other symptoms of anaphylaxis, immediate emergency care is required.

• Vasculitis. Vasculitis is an autoimmune disease that causes inflammation in blood vessels, which means blood vessels swell and thicken and can be seen beneath the skin. It’s an internal problem that sometimes manifests with rashes or bumps on the skin. Swelling makes it difficult for blood to flow through the blood vessels, which can damage organs or other tissues.

Skin Infections

Infections happen when pathogens get into the body and start multiplying. When your skin is scratchy and itchy, it can break and let germs enter your body. Some skin infections are topical, but others go deep into the skin. The most common pathogens to cause skin infections include:

• Bacteria: cellulitis, impetigo, boils, staphylococcal infections

• Viruses: shingles (herpes zoster); chicken pox; molluscum contagiosum; warts; measles; hand, foot and mouth disease; herpes simplex

• Fungi: athlete’s foot, yeast infection, ringworm, nail fungus, oral thrush, diaper rash

• Parasites: lice, bedbugs, scabies, cutaneous larva migrans Symptoms of skin infections vary depending on the type and cause of the infection, and whether you have a weakened immune system.

However, common symptoms include redness on pale skin or purple/darkened areas on dark skin; lesions that are flat or raised, bumpy or wart-like; itching; and pain and tenderness and skin that is warm to the touch. Symptoms of severe infection include pus, blisters, skin sloughing/breakdown, dark areas that can indicate necrosis (tissue death), pain and discoloration or widespread swelling. People who are older, have poor circulation, have diabetes, are malnourished, have excessive skin folds, must stay in one position for a long time (such as those who are paralyzed), have an immune system disease or are immunocompromised have an increased risk of skin infections. If you have a PI, you are at heightened risk for skin infections.

Special Considerations for People with PI

The skin has a lot to say about everyone’s health, but if you have a PI, you should pay particularly close attention to what your skin says. PI weakens your immune system, which makes it harder to fight off pathogens, and immune dysfunction can sometimes cause your body to mistakenly attack its own healthy cells (as in autoimmune skin diseases). As a result, you may tend to suffer from a wide variety of skin conditions. Allergic disorders, skin infections, autoimmune conditions, autoinflammation and cancer are often seen in PI. According to Edward Cowen, MD, senior clinician head at the National Institutes of Health Dermatology Consultation Service, visible skin problems can be important for diagnosing and managing health issues in PI patients. In fact, skin manifestations are often one of the first signs that point to a PI diagnosis. According to the International Patient Organisation for Primary Immunodeficiencies, “Up to 70 percent of people with a [PI] exhibit skin manifestations (such as bacterial, fungal [e.g., candidiasis], viral infections [e.g., molluscum and warts] or eczema), and these signs may be among the primary presenting symptomatology of a [PI].” If you have a PI, you should monitor your skin every day, prioritize sun protection and see a dermatologist at least once a year. Of primary concern: skin cancer. “One in three people of European descent will develop basal cell carcinoma, and the rate is higher in those with PI.” People with PI are also more susceptible to squamous cell carcinoma (an aggressive form of skin cancer).

Annual visits with a dermatologist can help identify skin cancer, as well as the following additional common skin conditions in PI:

• Dermatitis (skin inflammation). Dermatitis is one of the most common noninfectious skin manifestations in PI. Notably, PI patients who have eczema tend to develop food allergies, asthma and rhinoconjunctivitis (hay fever). Dermatitis is itchy; scratching itchy skin can break the skin, which can lead to infections on the skin or in the bloodstream.

• Autoimmune skin conditions. Psoriasis, lupus, sclero-derma, dermatomyositis, Behcet’s disease, pemphigus and pemphigoid are common in PI patients because immune system dysfunction sometimes causes the body to attack healthy skin cells.16

• Bacterial infections. Staphylococcus aureus-induced skin infections are the most common skin infections reported in people with PI, especially in those with leukocyte adhesion defects (LAD), chronic granulomatous disease (CGD), severe congenital neutropenia and hyper-IgE syndrome (HIES). Some bacterial infections are pyogenic, or pusproducing. (Pus is a sign of an immune response to an infection.) PIs such as CGD, LAD and HIES may present with pyogenic infections; infections may manifest as folliculitis (inflammation of follicles), abscesses (a localized collection of pus surrounded by inflamed tissue), furuncles (boils) or impetigo (red sores around the face that ooze and develop honey-colored crusts).

• Erythroderma. This is a condition with severe skin irritation causing redness of the skin and/or scaling involving more than 90 percent of the total body surface. Almost half of erythroderma in infancy is due to PI.

• Alopecia. Alopecia (hair loss) is the third most common skin condition in patients with PI.

• Fungal infections. Fungal infections in PI patients are linked with combined T- and B-cell immunodeficiencies such as severe combined immunodeficiency (SCID). In babies, SCID may present with persistent yeast infections in the mouth (thrush) and/or on a baby’s bottom.

• Viral infections. Herpes zoster, herpes simplex and human papillomavirus are typical in neutropenia and hypogammaglobulinemia due to deficiency or defects in CD4+ T cells and NK cells. Warts may indicate combined immune deficiency or WHIM syndrome.

Listen to Your Skin Some skin conditions are short-lived, minor irritations; others are chronic, extensive skin diseases that need medical attention and ongoing management. Some are noninfectious and only require comfort measures, while others are infectious and require treatment (antibiotics, antivirals, antifungals or antiparasitics) or even hospitalization.

One of the best things you can do to take care of your skin is to pay attention to what it is trying to tell you, especially if you have a PI, and talk to your doctor when you notice something new. Equally important: Prioritize sun protection. The best way to guard against skin cancer is to take precautionary measures to prevent it. See our product guide for sun protection recommendations.