en Dermathology Dermathology case report case report <span style="font-size:14px;"><span style="font-family:Times New Roman;"><span style="color:#0000ff;"><strong><em>Background: </em></strong></span>Waldenstrom macroglobulinemia is a lymphoplasmacytic lymphoma with elevated serum immunoglobulin M and multi-organ involvement. Primary systemic amyloidosis usually develops due to immunoglobulin light chains depositions in different organs due to an underlying gammopathy.<br> <span style="color:#0000ff;"><em><strong>Case presentation:</strong></em></span> Our patient was an 86-year-old man with macroglossia, ecchymotic patches and bullous lesions associated with a skin laxity on the periorbital, palmar, and glans penis areas. Skin biopsy confirmed dermal amyloid depositions. In serum immunofixation electrophoresis, prominent monoclonal immunoglobulin-M lambda light chains were detected associated with prominent lymphoplasmacytic infiltration in bone marrow biopsy which was diagnosed as Waldenstrom macroglobulinemia.<br> <span style="color:#0000ff;"><em><strong>Conclusion: </strong></em></span>Skin involvement presenting as cutaneous amyloidosis could be the first manifestation of Waldenstrom macroglobulinemia. We should think about an underlying gammopathy in an old patient with skin laxity and ecchymosis.</span></span> Primary systemic amyloidosis, Waldenstrom macroglobulinemia 340 342 http://caspjim.com/browse.php?a_code=A-10-658-4&slc_lang=en&sid=1 Rana Rafiei rafieirana2@gmail.com 100319475328460023919 100319475328460023919 Yes Skin Research Center, Dermatology Department, Guilan University of Medical Sciences, Rasht, Iran Hojat Eftekhari dr.he.derm@gmail.com 100319475328460023920 100319475328460023920 No Skin Research Center, Dermatology Department, Guilan University of Medical Sciences, Rasht, Iran Behnam Rafiee behnam2776@gmail.com‬‬‬‬‬‬‬‬‬‬‬‬‬‬‬‬‬‬‬‬‬‬‬‬‬‬ 100319475328460023921 100319475328460023921 No Department of Pathology, NYU Winthrop Hospital, 222 Station Plaza, No. 620, Mineola, NY 11501, USA