Blood Res 2014; 49(3): 146
Primary systemic amyloidosis with sole cutaneous involvement
Prakas Kumar Mandal1*, and Tuphan Kanti Dolai2

1Department of Pathology, I.P.G.M.E.& R., Kolkata-700020, India.

2Department of Hematology, N.R.S. Medical College, Kolkata-700014, India.

Correspondence to: Correspondence to Prakas Kumar Mandal, M.D., Department of Pathology, I.P.G.M.E.& R., 8C/1/N, Roy para road, First floor, Kolkata-700050, WB, India,
Published online: September 25, 2014.
© The Korean Journal of Hematology. All rights reserved.

cc This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.


A 60-year-old woman showed blackish pigmentation around both eyes for 2 years, with clinical examination revealing waxy hyperpigmentation and small nodules (A). Blood examination revealed Hb, 7.5 g/dL; WBC, 6.2×109/L; platelets, 210×109/L; corrected reticulocytes, 1.4%; plasma fasting glucose, 81 mg/dL; serum urea, 26 mg/dL; creatinine, 0.9 mg/dL; total protein, 8.92 g/dL; albumin, 3.96 g/dL; globulin, 4.96 g/dL; albumin/globulin ratio, 0.8:1; calcium, 12.7 mg/dL; and 24-hour urinary protein, 75 mg. A peripheral blood smear showed normocytic, normochromic RBCs with rouleaux formation. Serum protein electrophoresis showed a monoclonal band in the gamma globulin region; serum immunofixation electrophoresis showed monoclonal lambda light chain immunoglobulin. Serum kappa and lambda light chain levels were 6.34 and 626.24 mg/L, respectively, with an altered kappa/lambda ratio (0.01; reference: 0.26-1.65). Radiographic results were negative. Abdominal ultrasonography revealed normal renal corticomedullary differentiation; echocardiography findings were normal. Nerve conduction velocity was normal in all limbs. Punch biopsies from periocular lesions showed amorphous pale pink dermal deposits (hematoxylin-eosin staining; B, lower magnification; C, higher magnification); Congo red staining showed apple-green birefringence on polarizing microscopy. The bone marrow plasma cell proportion was 49%. Conventional cytogenetics indicated a normal karyotype. Thus, primary systemic amyloidosis may present as isolated skin lesions.


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