Sjogren’s syndrome is an autoimmune disorder that affects the moisture-producing glands in the body. This includes the tear glands and salivary glands, leading to the two hallmark symptoms of Sjogren’s: dry eyes and dry mouth. However, Sjogren’s syndrome can impact other parts of the body as well, including the skin. Individuals with Sjogren’s often experience various skin manifestations due to the lack of moisture and lubrication. This article will provide an overview of the common skin symptoms associated with Sjogren’s syndrome and what Sjogren’s skin typically looks like.
Dry, Itchy Skin
One of the most common skin complaints in Sjogren’s patients is xerosis, which refers to dry, itchy skin. With Sjogren’s, the sebaceous glands in the skin produce less sebum, the natural oils that help keep skin moisturized. This leads to skin that is excessively dry, scaly, and itchy. Sjogren’s patients often experience xerosis all over the body, but especially on the arms, legs, torso, and face. The skin may look cracked, flaky, rough, and red from excessive scratching. Severely dry skin can also progress to eczema in Sjogren’s patients.
Skin Rashes
Skin rashes are another common symptom of Sjogren’s syndrome. Some Sjogren’s patients develop chronic urticaria, which causes red, itchy welts on the skin that come and go. Rashes associated with Sjogren’s may be due to the autoimmune attack on the skin itself or skin irritation from severe dryness. Some other skin rashes seen in Sjogren’s include:
- Erythema annulare – Red ring-shaped patches
- Hypergammaglobulinemic purpura – Tiny red dots on the skin caused by blood vessel leakage
- Leukocytoclastic vasculitis – Red spots on the skin resulting from inflamed blood vessels
- Palpable purpura – Purple bruise-like spots on the skin
These rashes tend to come and go and are often itchy or painful. They typically occur on the lower legs but can appear anywhere on the body.
Photosensitivity
Many individuals with Sjogren’s syndrome find their skin has increased sensitivity to sunlight. When photosensitivity occurs with Sjogren’s, exposure to ultraviolet (UV) rays causes a skin rash or irritation. This photosensitive reaction can occur even with minimal sun exposure. The rash often shows up as red, bumpy, itchy patches on sun-exposed areas like the face, back of hands, and arms. To avoid this reaction, Sjogren’s patients must take care to limit sun exposure and wear protective clothing and sunscreen.
Vasculitic Ulcers
Vasculitis refers to inflammation of the blood vessels. About 20% of Sjogren’s patients experience vasculitis, which can affect the skin. This leads to vasculitic ulcers, which are open sores on the skin surface caused by damaged blood vessels underneath. These sores typically occur on the lower legs and ankles. Vasculitic ulcers may start out looking like a bruise or reddish-purple dots but can progress to an open wound if the damaged blood vessels do not heal.
Calcinosis Cutis
A small percentage of Sjogren’s patients develop calcinosis cutis – deposits of calcium in the skin. This causes firm, chalky white lumps to form, typically on the fingers, hands, elbows, and knees. While not painful, these calcium deposits can break open and leak a white chalky discharge, causing damage to the surrounding skin. Calcinosis cutis occurs due to damaged blood vessels in these localized areas.
Raynaud’s Phenomenon
Raynaud’s phenomenon is a condition that causes the fingers and toes to become abnormally cold and numb in response to cold exposure or stress. It occurs due to vessels in the extremities going into vasospasm, drastically reducing blood flow. Raynaud’s often causes color changes in the skin of the digits, such as from white to blue to red. About 20% of Sjogren’s patients have Raynaud’s phenomenon.
angular cheilitis
Angular cheilitis is a condition characterized by red, cracked sores in the corners of the mouth. It is caused by fungal infection or irritation at the mouth corners. Because Sjogren’s results in dry mouth, angular cheilitis is common in these patients due to lack of protective saliva. The corners of the mouth appear red, swollen, and cracked.
Pruritus Ani
Pruritus ani refers to itching around the anus. It is another skin irritation issue that can occur in Sjogren’s patients, especially women. The itching results from chronic diarrhea associated with Sjogren’s drying out the skin around the anus. This leaves the sensitive area prone to irritation.
Vitiligo
Vitiligo causes depigmentation of patches of skin. It occurs when the cells responsible for skin pigment are destroyed. Vitiligo is an autoimmune condition and is more common in Sjogren’s patients because Sjogren’s is also autoimmune. Patches of light, white skin will develop due to loss of melanin. Common locations include the face, hands, arms, and torso.
Mucosal Changes
In addition to skin dryness, individuals with Sjogren’s syndrome often develop changes in their mucosal tissues inside the body. This includes the moist tissues lining areas like the mouth, eyes, nose, throat, vagina, and gastrointestinal tract. With inadequate moisture, the mucosa can become irritated and inflamed. Symptoms include mouth sores, dry cough, hoarse voice, swollen parotid glands, dry nasal passages, vaginal dryness and irritation, and esophageal reflux.
Summary of Sjogren’s Skin Manifestations
| Skin Manifestation | Description |
|---|---|
| Dry, itchy skin (xerosis) | Rough, flaky, scaly skin anywhere on body |
| Skin rashes | Red, itchy rashes and hives on skin |
| Photosensitivity | Skin irritation/rash after sun exposure |
| Vasculitic ulcers | Ulcerated sores on lower legs |
| Calcinosis cutis | Hard white papules and nodules on skin |
| Raynaud’s phenomenon | Fingers/toes abnormally numb and cold |
| Angular cheilitis | Cracked sores at corners of mouth |
| Pruritus ani | Anal itching |
| Vitiligo | Patches of skin losing pigment |
| Mucosal changes | Dry, inflamed mucosal tissues in body |
Diagnosing Sjogren’s Skin Problems
Diagnosing Sjogren’s skin issues involves a skin examination by a dermatologist. They will evaluate the appearance and distribution of any skin lesions. The dermatologist will also review the person’s full medical history, including symptoms of dry eyes and dry mouth. Blood tests may be ordered to check for Sjogren’s antibodies. A lip biopsy of the salivary glands may also help confirm Sjogren’s syndrome. Skin biopsies are sometimes performed on skin lesions to help determine the cause. Once Sjogren’s is diagnosed, the various skin problems that often accompany it make more sense and can be treated appropriately.
Treatment for Sjogren’s Skin Symptoms
While Sjogren’s syndrome itself has no cure, many of the associated skin problems can be effectively managed with treatment.
Some key treatments for Sjogren’s skin issues include:
- Use of thick, rich moisturizing creams and ointments to improve skin hydration
- Prescription steroid creams to reduce skin inflammation and itch
- Oral antihistamines to control itching
- Topical immune modulating creams for skin rashes
- Antifungal creams for angular cheilitis
- Avoiding irritants like scented products and wool
- Wearing gloves and socks in cold weather if Raynaud’s present
- Avoiding sun exposure and using sunscreen
- Using humidifiers to add moisture to indoor air
For more severe skin manifestations like vasculitic ulcers, additional medications like immunosuppressants, biologics, or Rituximab may be used. Lifestyle measures like quitting smoking, managing stress, and eating healthy can help reduce flares. Proper skin care and moisture are key to managing Sjogren’s skin problems.
Conclusion
Sjogren’s syndrome can significantly impact the skin, causing a range of problems like xerosis, rashes, photosensitivity, Raynaud’s phenomenon, and skin ulcers. The hallmark of Sjogren’s skin is dryness, which leads to itching, irritation, and sensitivity. Proper skin care, moisture replacement, sun protection, and certain medications can help control the skin manifestations associated with this condition. Paying attention to skin changes and reporting them promptly to a doctor allows for better management. With proper treatment, those with Sjogren’s can keep their skin healthy and comfortable despite the chronic dryness.