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Dear Editor:
Altman and Winkelmann
1 first described diffuse normolipemic plane xanthomatosis (DNPX) in 1962. Patients with DNPX usually present with yellowish plaques that characteristically appear on the periorbital region, neck, and flexural folds; plasma lipid levels are normal
12. Relationships with underlying hematological diseases, particularly multiple myeloma and other types of monoclonal gammopathy, have frequently been suggested
34.
A 69-year-old woman presented with a 10-year history of slowly increasing yellow skin lesions on the whole body. She showed symmetrically distributed well-demarcated yellow plaques in both periorbital areas and multiple yellow patches and papules in the flexural area, including the axilla and the inguinal region (
Fig. 1). The patient did not complain of any systemic symptoms. Laboratory examinations revealed iron deficiency anemia and hypoalbuminemia. Plasma lipid and triglyceride levels were within normal ranges. Urinalysis revealed overt proteinuria and hematuria. Serum electrophoresis showed a monoclonal peak in 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.
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 are phagocytosed by macrophages, which finally appear as foam cells
34. Several cases of DNPX associated with monoclonal gammopathy have been reported to date
345, 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 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 hematological or lymphoproliferative diseases
5. Therefore, in patients with DNPX, skin biopsy and hematological evaluation should be performed, and close follow-up with periodic laboratory testing is recommended.
Figures and Tables
Fig. 1
(A) Symmetrically distributed yellow plaques on both periorbital areas. (B) Yellow patches, papules on the axilla. We received the patient's consent form about publishing all photographic materials.
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).
ACKNOWLEDGMENT
This work was supported by the Soonchunhyang University Research Fund.
References
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