Cutaneous features and diagnosis of primary Sjögren syndrome: An update and review

J Am Acad Dermatol. 2018 Oct;79(4):736-745. doi: 10.1016/j.jaad.2018.05.021. Epub 2018 May 19.

Abstract

Sjögren syndrome (SS) is an autoimmune connective tissue disorder (CTD) that principally affects the lacrimal and salivary glands. Although SS is 1 of the 3 most common autoimmune CTDs alongside systemic lupus erythematosus and progressive systemic sclerosis, it is the least researched CTD overall. SS poses a particular diagnostic challenge because it shares multiple clinical and immunologic features with other CTDs. However, there are some characteristic cutaneous clinical features that can precede the well-known sicca symptoms by years. By familiarizing themselves with these clinical features and having a high suspicion for SS, dermatologists can play an important role in the early diagnosis and treatment of this disease.

Keywords: Sjögren syndrome; annular erythema; anti-Ro (SS-A); cutaneous vasculitis; localized cutaneous nodular amyloidosis; sicca.

Publication types

  • Review

MeSH terms

  • Autoimmune Diseases / diagnosis*
  • Autoimmune Diseases / therapy
  • Biopsy, Needle
  • Connective Tissue Diseases / immunology
  • Connective Tissue Diseases / pathology*
  • Connective Tissue Diseases / therapy
  • Dermatologic Agents / therapeutic use
  • Diagnosis, Differential
  • Female
  • Humans
  • Immunohistochemistry
  • Immunosuppressive Agents / therapeutic use
  • Lupus Erythematosus, Cutaneous / immunology
  • Lupus Erythematosus, Cutaneous / pathology*
  • Lupus Erythematosus, Cutaneous / therapy
  • Male
  • Prognosis
  • Risk Assessment
  • Sjogren's Syndrome / immunology
  • Sjogren's Syndrome / pathology*
  • Sjogren's Syndrome / therapy*
  • Treatment Outcome

Substances

  • Dermatologic Agents
  • Immunosuppressive Agents