Periorbital papules as a presenting sign in multiple myeloma with AL amyloidosis

Description

A 67-year-old woman of Indian heritage with no significant medical history presented with a 9-month history of progressive periorbital lesions consisting of coalescing pigmented papules (figure 1A). On systems review she reported concurrent diarrhoea and weight loss. She was not in overt heart failure and had no other cutaneous lesions or macroglossia.

Figure 1

(A) Confluent periorbital lesions. (B) Deposition of pink amorphous material on histological section. (C) CD 138 positive plasma cell infiltrate on bone marrow trephine section.

A biopsy of the lesions identified amorphous amphophilic material in the upper dermis (figure 1B), with positive staining for crystal violet, consistent with amyloid deposition. Further investigations demonstrated markedly elevated serum light chains at 5423 mg/L with a ratio of <0.01 and a free light chain paraprotein of <2 g/L with concurrent immuneparesis. A bone marrow biopsy demonstrated replacement...

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