Localized cutaneous nodular amyloidosis associated with Sjögren’s syndrome

A 61-year-old female patient with primary Sjögren’s syndrome presented with several variously sized hemorrhagic nodules and plaques over her nape and upper back without previous trauma history [Figure 1a]. Another raised waxy yellowish asymptomatic plaque was also found on her left cheek [Figure 1b]. No signs of macroglossia or Raynaud’s phenomenon were observed. Histological analysis of back and face lesions revealed amorphous eosinophilic deposition in the dermis [Figure 2a] with apple-green birefringence in Congo red stain [Figure 2b]. The immunohistochemical staining for κ and λ immunoglobulin light chains was negative. The laboratory findings demonstrated elevated ESR, ANA, RA factor, IgG, IgA, anti-Ro, and anti-La antibodies. M protein was not detected in serum immunoelectrophoresis. Bence-Jones protein was negative. The diagnosis of localized cutaneous nodular amyloidosis (LCNA) associated with Sjogren’s syndrome was made. She was followed up regularly without any further treatment for her cutaneous lesions. The unique yellowish waxy plaques with hemorrhage in a patient with Sjögren’s syndrome should raise the differential diagnosis of LCNA.

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Figure 1:

(a) Several variously sized hemorrhagic nodules and plaques over the nape and upper back. (b) A raised waxy yellowish asymptomatic plaque was also found on the left cheek.

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Figure 2:

(a) Histological analysis revealed amorphous eosinophilic deposition in the dermis (H&E). (b) Apple-green birefringence in Congo red stain.

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