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A case of diffuse plane normolipemic xanthomatosis associated with pancytopenia and monoclonal gammopathy
Author(s) -
KOUROU Kazuhiro,
SUGA Yasushi,
MURAMATSU Shigenori,
YAGUCHI Hitoshi,
OGAWA Hideoki
Publication year - 2006
Publication title -
the journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.9
H-Index - 65
eISSN - 1346-8138
pISSN - 0385-2407
DOI - 10.1111/j.1346-8138.2006.00013.x
Subject(s) - pancytopenia , pathology , monoclonal gammopathy of undetermined significance , medicine , biopsy , monoclonal gammopathy , gammopathy , bone marrow , dermis , dermatology , monoclonal , monoclonal antibody , immunology , antibody
We report a case of diffuse plane normolipemic xanthomatosis (DPNX) which showed poorly demarcated, uncommon, yellow macules symmetrically distributed on the nape, axillae and inguinal folds accompanied by severe, persistent itching. Histopathological and ultrastructural studies of skin biopsy specimens revealed the existence of some foamy cells and the deposition of neutral fat in the upper papillary dermis. Laboratory investigations and bone marrow aspirate smears showed that our patient had myelodysplastic syndrome (MDS) associated with pancytopenia and monoclonal gammopathy of undetermined significance. Because our patient had neither a malignant hematological disorder nor a severe systemic disease, monoclonal gammopathy might explain the pathogenesis of DPNX in the present case.