Sjögren’s syndrome is an autoimmune condition where the immune system attacks moisture-producing glands, primarily the tear and salivary glands, leading to chronic dryness of the eyes and mouth (sicca symptoms). Although dryness is the most recognized feature, the immune dysfunction often affects other organs, including the skin. Skin manifestations, such as distinct types of rashes, occur in nearly half of people with Sjögren’s. These rashes result from inflammation within the small blood vessels or the presence of specific autoantibodies, and they vary significantly in appearance and severity.
The Most Common Sjogren’s Skin Manifestation
The most characteristic rash associated with Sjögren’s syndrome is cutaneous vasculitis, which is inflammation in the small blood vessels. This immune-mediated damage occurs when immune complexes deposit within the vessel walls, causing leakage and local inflammation. The visual result is often a rash called palpable purpura, a term describing its appearance and feel.
Palpable purpura is distinguished by raised, reddish-purple spots or bumps. When pressed, these lesions do not lighten or blanch, indicating blood leakage from damaged vessels into the surrounding skin tissue. The lesions typically range from tiny dots to larger patches, often appearing in clusters.
Less Common but Severe Skin Manifestations
Other distinct rashes are associated with Sjögren’s, often indicating different underlying immune mechanisms. One manifestation is annular erythema, which presents as ring-shaped or circular lesions. These lesions are typically red or pink with a slightly raised edge, sometimes resembling a target.
Annular erythema is strongly linked to the presence of specific autoantibodies, notably anti-Ro/SSA, and is considered a form of subacute cutaneous lupus erythematosus. Another less frequent presentation is hypergammaglobulinemic purpura, caused by abnormally high levels of immune proteins (gamma globulins). This purpura appears as small, recurrent, purplish-red spots, usually on the lower legs, and is a non-vasculitic form of blood vessel leakage.
Location and Associated Symptoms
The location of a Sjögren’s rash is often determined by gravity and exposure, providing important diagnostic clues. Vasculitic rashes most commonly appear on the lower extremities, specifically the ankles and lower legs. This is because the small blood vessels in this area are under the highest pressure, making them more susceptible to inflammation and leakage.
Rashes related to photosensitivity, such as annular erythema, commonly appear on sun-exposed areas like the face, neck, upper chest, and arms. The skin lesions can be accompanied by sensations including pain, burning, or intense itching (pruritus). In severe vasculitis cases, damage to the blood vessels can lead to skin breakdown, resulting in small ulcers or open sores, most often around the ankles.
Managing Sjogren’s Related Rashes
Effective management of a Sjögren’s rash depends on its specific type and severity, requiring a joint approach between a rheumatologist and a dermatologist. For milder, non-ulcerative rashes, general skin care is important, including avoiding hot water and harsh soaps. Applying thick, non-perfumed moisturizers immediately after a short, lukewarm shower helps maintain skin barrier integrity.
Sun protection is required, especially for those with photosensitive rashes, involving broad-spectrum sunscreen and protective clothing. Medical treatment for widespread or symptomatic rashes often includes anti-malarial drugs, such as hydroxychloroquine, to reduce systemic inflammation. For significant or ulcerating vasculitis, stronger immunosuppressive medications or oral corticosteroids may be prescribed to control blood vessel inflammation and prevent potential organ damage.