Can Sjögren's syndrome cause rashes?

Can Sjögren's Syndrome Cause Rashes?

Yes, Sjögren's syndrome causes cutaneous manifestations in approximately 50% of patients, ranging from nonspecific findings like dry skin and pruritus to clinically significant rashes including palpable purpura from cutaneous vasculitis, erythema annulare, and urticaria-like lesions. 1, 2

Common Skin Manifestations

The dermatologic involvement in Sjögren's syndrome includes several distinct patterns:

Nonspecific Manifestations

• Xerosis (dry skin) is the most common cutaneous finding, affecting many patients with Sjögren's syndrome 1, 3
• Pruritus occurs frequently and may be severe enough to cause diffuse scratching-induced lesions 1, 4
• Angular cheilitis presents as painful sores or red patches at the corners of the mouth 5, 3
• Eyelid dermatitis can develop as part of the periocular dryness complex 3, 4

Clinically Significant Rashes

Cutaneous vasculitis is the most important skin manifestation to recognize, as it carries prognostic significance:

• Presents most commonly as palpable purpura, typically on the lower extremities 2, 3
• May also manifest as nonpalpable purpura or urticaria-like lesions 2, 4
• This finding is of paramount clinical importance because it confers increased risk for multisystem vasculitis and non-Hodgkin lymphoma 2

Erythema annulare is a distinctive annular erythematous rash that can occur in primary Sjögren's syndrome 3, 4

Other specific manifestations include:

• Livedo reticularis, which may occur even without vasculitis 4
• Pernio-like lesions have been reported 3
• Localized nodular cutaneous amyloidosis, possibly representing lymphoproliferative disease 4

Critical Clinical Pearls

The skin manifestations are often underrecognized and underdiagnosed because they tend to be overshadowed by the more prominent sicca symptoms (dry eyes and dry mouth) and are generally less severe than oral, ocular, or musculoskeletal symptoms 1, 2

However, certain cutaneous findings—particularly palpable purpura indicating cutaneous vasculitis—should prompt immediate concern as they signal increased risk for:

• Life-threatening multisystem vasculitis 2
• Non-Hodgkin lymphoma (approximately 5% lifetime risk in Sjögren's syndrome) 5, 2

Diagnostic Approach When Rash is Present

When evaluating a patient with suspected Sjögren's syndrome and cutaneous manifestations:

• Perform careful skin examination looking specifically for palpable purpura, erythema annulare, and signs of vasculitis 2, 3
• Assess for systemic dryness including xerophthalmia, xerostomia, and xeroderma 1, 4
• Order complete serological panel including anti-SSA/Ro, anti-SSB/La, rheumatoid factor, and antinuclear antibody 6, 5
• Consider skin biopsy if vasculitis is suspected, as this has important prognostic implications 2
• Check inflammatory markers and complement levels (particularly C4), as decreased C4 at diagnosis indicates higher lymphoma risk 6, 5

Management Implications

Mandatory rheumatology referral is essential when Sjögren's syndrome is diagnosed, particularly if cutaneous vasculitis is present, due to the increased risk of systemic complications and lymphoproliferative disorders 5, 2

For symptomatic management of skin manifestations:

• Patient education to avoid alcohol and tobacco smoking 4
• Topical emollients for xerosis 4
• Treatment approach is similar regardless of the specific cause of dryness 4

For vasculitic manifestations, systemic immunosuppressive therapy may be required and should be coordinated with rheumatology 5