A skin biopsy from her axilla showed scattered foamy histiocytes in the upper and mid dermis, with an admixture of histiocytes and lymphocytes (Fig. and hematuria. Serum electrophoresis showed a monoclonal peak in Ginsenoside Rh2 the gamma region and immunoglobulin G-lambda monoclonal gammopathy. An echocardiogram showed congestive heart failure and a supracardinal lymph-node biopsy revealed amyloid deposition. Renal biopsy demonstrated amyloid light chain-related amyloidosis. Bone marrow biopsy revealed aggregates of plasma cells with lambda monoclonality. Based on these findings, the patient was diagnosed with multiple myeloma. A skin biopsy from her axilla showed scattered foamy histiocytes in the upper and mid dermis, with an admixture of histiocytes and lymphocytes (Fig. 2A). Immunohistochemical staining was diffusely positive for CD68 (Fig. 2B). Dense staining throughout the entire dermis for the immunoglobulinlambda light chain was also observed (Fig. 2C). These findings were consistent with DNPX. The papillary dermis contained globular deposits of homogenous, faintly eosinophilic materials (Fig. 2D). However, they showed no definite affinity to congo red. Open in a separate window Fig. 1 (A) Symmetrically distributed yellow plaques on both periorbital areas. (B) Yellow patches, papules on the axilla. We Ginsenoside Rh2 received the patient’s consent form about publishing all photographic materials. Open in a Ginsenoside Rh2 separate window Fig. 2 (A) Scattered foamy histiocytes are observed in the upper and mid dermis, with an admixture of histiocytes and lymphocytes (H&E, 200). (B) Immunohistochemical staining was diffusely positive for CD68 (CD68, 100). (C) Dense staining throughout the entire dermis for the immunoglobulin-lambda light chain is noted (lambda chain, 100). (D) Globular deposits of homogenous, faintly eosinophilic materials in the papillary dermis (H&E, 200). The pathogenesis of DPNX remains unclear. The previously suggested pathogenesis of DNPX is that monoclonal immunoglobulins bind to circulating low-density lipoproteins in plasma and form complexes around vessels in the skin, where they Ginsenoside Rh2 are phagocytosed by macrophages, which finally appear as foam cells3,4. Several cases of DNPX associated with monoclonal gammopathy have been reported to date3,4,5, but this report is the first to describe dense lambda immunoglobulin deposition with infiltration of histiocytes in the entire dermis, as revealed by skin biopsy. Furthermore, we cautiously suggest the coexistence of cutaneous amyloidosis, which is another unique finding of this report, based on Ginsenoside Rh2 the observation of globular deposits of eosinophilic materials in the papillary dermis on histological examination. Cutaneous amyloid deposition may exist in this patient, considering that it is one of the symptoms of multiple myeloma, and the patient also had accompanying cardiac and renal amyloidosis. Although the globular deposits show no definite affinity to congo red, inadequate tissue quantity and staining might have caused a false negative result or decreased sensitivity. Although not all xanthomas are associated with systemic diseases, DNPX may appear as the first symptom of underlying malignant Sox17 hematological or lymphoproliferative diseases5. Therefore, in patients with DNPX, skin biopsy and hematological evaluation should be performed, and close follow-up with periodic laboratory testing is recommended. ACKNOWLEDGMENT This work was supported by the Soonchunhyang University Research Fund. Footnotes CONFLICTS OF INTEREST: The authors have nothing to disclose..