Nodular localized cutaneous amyloidosis in an adult patient with Sjögren’s syndrome

A 50-year-old woman presented with clustered, yellowish, soft, waxy nodules 0.2–0.4 cm in diameter on the right middle abdomen for 5 years (Fig. 1A). Atrophic wrinkles developed on the overlying skin with striae distensae around the lesions. A skin biopsy showed massive deposition of amorphous eosinophilic material with irregular clefts in the entire dermis as well as sparse plasma cell infiltrate (Fig. 1B). Congo red staining was positive. Immunohistochemical examination demonstrated negative staining for kappa and lambda light chains. She was diagnosed with primary SS at the age of 48 years. SS was well controlled with 10 mg/day methylprednisolone. She had positive Ro/SSA and La/SSB antibodies and no abnormalities of other laboratory findings. A diagnosis of nodular localized cutaneous amyloidosis (NLCA) was made. NLCA is one type of cutaneous amyloidosis. Approximately 25% of NLCA cases have been associated with SS [1]. NLCA in SS has been hypothesized to be from benign clonal plasma cell proliferation in the skin. The concurrence of these two diseases may represent a part of the spectrum of lymphoproliferative diseases related to SS [1]. This case highlights that the associated SS should be evaluated after establishing the diagnosis of NLCA and long-term follow-up is warranted.

Fig. 1

NLCA in a 50-year-old woman

(A) Multiple yellowish, soft, waxy nodules on the right abdomen. (B) Histopathology shows flattened epidermis and massive deposition of amorphous eosinophilic material with irregular clefts in the entire dermis.

Open in new tabDownload slide

Funding: This study was supported by the CAMS Innovation Fund for Medical Sciences (2016-I2M-1-003) and the National Natural Science Foundation of China (81703148).

Disclosure statement: The authors have declared no conflicts of interest.

Reference

Author notes