A case of diffuse plane normolipemic xanthomatosis associated with pancytopenia and monoclonal gammopathy.
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.
Department of Dermatology, Juntendo University School of Medicine, Tokyo, Japan.
Kazuhiro
K
Kourou
Yasushi
Y
Suga
Shigenori
S
Muramatsu
Hitoshi
H
Yaguchi
Hideoki
H
Ogawa
D000368:Aged; D001365:Axilla / Q000473:pathology; D003937:Diagnosis, Differential; D006801:Humans; D008297:Male; D008998:Monoclonal Gammopathy of Undetermined Significance / Q000097:blood / Q000150:complications / Q000175:diagnosis*; D010198:Pancytopenia / Q000097:blood / Q000150:complications / Q000175:diagnosis*; D012871:Skin Diseases / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D014973:Xanthomatosis / Q000150:complications / Q000175:diagnosis* / Q000473:pathology