Clinical Reviews in Allergy & Immunology

, Volume 53, Issue 3, pp 357–370 | Cite as

Cutaneous and Mucosal Manifestations of Sjögren’s Syndrome

  • Elena Generali
  • Antonio Costanzo
  • Carlo Mainetti
  • Carlo SelmiEmail author


Sjögren’s syndrome is currently considered an “autoimmune epithelitis,” as exocrine glands, especially salivary and lacrimal, are progressively destructed by an immune-mediated process associated with specific serum autoantibodies and local lymphocyte infiltrate. Xerostomia remains a key complain in patients with Sjögren’s syndrome but should be evaluated also for other causes such as xerogenic medications, followed by radiation and chemotherapy for head and neck cancers, hormone disorders, infections, or other connective tissue diseases. Further, xerophtalmia (also known as dry eye) frequently associated with keratoconjunctivitis sicca cumulatively affects approximately 10–30% of the general population with increasing incidence with age and is more frequently secondary to non-autoimmune diseases. On the other hand, numerous patients with Sjögren’s syndrome manifest signs of systemic dryness involving the nose, the trachea, the vagina, and the skin, suggesting that other glands are also affected beyond the exocrine epithelia. Skin involvement in Sjögren’s syndrome is relatively common, and various manifestations may be present, in particular xeroderma, eyelid dermatitis, annular erythema, and cutaneous vasculitis. Additional skin non-vasculitic manifestations include livedo reticularis which may occur in the absence of vasculitis, and localized nodular cutaneous amyloidosis possibly representing lymphoproliferative diseases related to Sjögren’s syndrome. The treatment of skin and mucosal manifestations in Sjögren’s syndrome is similar regardless of the cause, starting from patient education to avoid alcohol and tobacco smoking and to pursue dental hygiene. In conclusion, a strict collaboration between the dermatologist and the rheumatologist is essential in the adequate management of Sjögren’s syndrome skin and mucosal manifestations.


Autoimmunity Connective tissue disease Sicca syndrome Vasculitis Antinuclear antibody B cell 



Sjögren’s syndrome


Systemic lupus erythematosous


Rheumatoid arthritis


Systemic sclerosis


Primary biliary cholangitis


Human leukocyte antigen


Interferon regulatory factor 5


Signal transducer and activator of transcription 4






Tumor necrosis factor


T helper


Salivary gland epithelial cells


Biliary epithelial cells


Antigen-presenting cells


Transforming growth factor beta


Natural killer


Immunoglobulin G


Immunoglobulin M


B cell-activating factor


Antinuclear antibodies


Pathogen-associated molecular patterns


Pattern recognition receptors


Toll-like receptors


Nucleotide-binding oligomerization domain


Rheumatoid factor


Anti-centromere antibodies


Centromeric protein


Anti-mitocondrial antibodies


Anti-cyclic citrullinated peptide


Fragment crystallizable region


Tear breakup time


Subacute cutaneous lupus erythematosus


European League Against Rheumatism Sjögren’s syndrome disease activity index


Atopic dermatitis


Annular erythema




European League Against Rheumatism


Anti-neutrophil cytoplasmic antibody




Disease-modifying antirheumatic drugs


B lymphocyte stimulator


Intravenous immunoglobulins


Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflict of interest.



Ethical Approval and Informed Consent

No ethics issues are raised relevant to this review article.


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Copyright information

© Springer Science+Business Media, LLC 2017

Authors and Affiliations

  1. 1.Division of Rheumatology and Clinical ImmunologyHumanitas Research HospitalMilanItaly
  2. 2.Division of DermatologyHumanitas Research HospitalMilanItaly
  3. 3.Humanitas UniversityMilanItaly
  4. 4.Dermatologia Ente Ospedaliero Cantonale, Ospedale Regionale BellinzonaBellinzonaSwitzerland
  5. 5.BIOMETRA DepartmentUniversity of MilanMilanItaly

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