Cutaneous Septic Embolism Presenting as Erythematous Plaques

392 Ann Dermatol Received June 12, 2012, Revised November 8, 2012, Accepted for publication November 22, 2012 Corresponding author: Javier Galve, Department of Dermatology, Hospital Clínic, C/ Villarroel 170, 08036 Barcelona, Spain. Tel: 34-93-2275400 (Ext 2358), Fax: 34-93-2275438, E-mail: [email protected] This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http:// creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. light-chains (Fig. 1F, no kappa light-chains were found: see Fig. 1E); hence, systemic immunoglobulin light-chain amyloidosis was suspected. In electron microscopy, amyloid fibrils were seen (Fig. 1G, H). She received bortezomib/dexamethasone, and it was planned to induce remission for autologous stem cell therapy with lenalidomide/dexamethasone. She died from amyloid-induced heart failure prior to the planned treatment. Typically, amyloid purpura occurs above the nipple-line, mostly on the head and neck, and particularly on the eyelids. Among the suspected reasons for dermatorrhagia are that factor X is decreased by binding to amyloid fibrils, and that amyloid deposits in blood vessel walls increase vessel fragility. As purpura may be among the first signs of systemic amyloidoses, it is of utmost importance for dermatologists to keep this sign in mind. A suspected diagnosis of amyloidosis may be the starting point for an interdiscipilinary treatment regimen as different organs may be involved. Furthermore, it is crucial to treat the underlying cause (e.g., multiple myeloma, plasmocytoma, renal insufficiency with hemodialysis). However, there are also a couple of hereditary systemic amyloidoses with cutaneous involvement, e.g., Meretojaʼs syndrome (i.e. gelsolin amyloidosis). Future treatments with siRNAs or antiamyloid antibodies are in the pipeline, and we will see which ones make their way from bench to bedside. REFERENCES 1. Eder L, Bitterman H. Image in clinical medicine. Amyloid purpura. N Engl J Med 2007;356:2406. 2. Schreml S, Szeimies RM, Vogt T, Landthaler M, Schroeder J, Babilas P. Cutaneous amyloidoses and systemic amyloidoses with cutaneous involvement. Eur J Dermatol 2010;20:152-160. 3. Falk RH, Comenzo RL, Skinner M. The systemic amyloidoses. N Engl J Med 1997;337:898-909. 4. Clos AL, Lasagna-Reeves CA, Wagner R, Kelly B, Jackson GR, Kayed R. Therapeutic removal of amyloid deposits in cutaneous amyloidosis by localised intra-lesional injections of anti-amyloid antibodies. Exp Dermatol 2010;19:904-911. 5. Hovey BM, Ward JE, Soo Hoo P, O’Hara CJ, Connors LH, Seldin DC. Preclinical development of siRNA therapeutics for AL amyloidosis. Gene Ther 2011;18:1150-1156.