Nodular Amyloidosis Associated with Scleroderma

Final Diagnosis: Kalla Light Chain Restricted Nodular Amyloidosis

Diagnosis comment: The patient's biopsy confirms the diagnosis of nodular amyloidosis. The nodular amyloidosis in this case is associated with kappa light chain restricted plasmacytic infiltrates, hence defining light chain associated amyloidosis and representing a form of post-germinal center B-cell lymphoproliferative disease.
In this case the patient appears to have a classic presentation of light chain associated nodular amyloidosis in the setting scleroderma.

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Slide 1: There is an extensive pattern of deposition of pink amorphous material within the dermis.

Slide 2: The deposits have a glassy eosinophilic quality with clefting demonstrating both a ropey and nodular pattern with areas of dystrophic calcification.

Slide 3: The amyloid is extensively highlighted by the Congo red stain.

Slide 4: The material is also metachromatic on the crystal violet preparation.

Slide 5: A few scattered plasma cells are noted. In examining the plasma cells they are very numerous. They are arranged around vessels primarily.

Slide 6: There is extensive immunoreactivity of the plasma cells for kappa.

Slide 7: A few plasma cells are highlighted by lambda. However, the kappa to lambda ratio would indicate kappa light chain restriction being greater than 5:1.