Which cutaneous findings suggest Sjögren syndrome?

Updated: Mar 05, 2021
  • Author: Sriya K Ranatunga, MD, MPH; Chief Editor: Herbert S Diamond, MD  more...
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Answer

Patients with Sjögren syndrome can develop a nonpalpable or palpable, vasculitic purpura, with lesions that are typically 2-3 mm in diameter and located on the lower extremities. The lesions, which can ulcerate, occur most often in patients with hypergammaglobulinemia or cryoglobulinemia. [36, 35]

Annular erythema with scales, localized especially on the face and neck, is recognized as a cutaneous manifestation of Sjögren syndrome. The patches are recurrent and resolve without hyperpigmentation; no photosensitivity is observed. [55]  Annular erythema is a common cutaneous manifestation in Japanese and other Asian patients; however, it is rarely seen in white patents. [56]

In Japanese patients with Sjögren syndrome, annular erythema is divided into the following 3 types:

  • Sweet disease–like annular erythema with an elevated border
  • Subacute cutaneous lupus erythematosus–like, marginally scaled erythema
  • Papular erythema

Those lesions bear some clinical similarities to the annular lesions of subacute cutaneous lupus erythematosus, but their histopathologic features are distinct from those of subacute cutaneous lupus erythematosus. Significant mucin depositions are observed.


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