Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026986 (myelodysplastic syndrome)
 14,926 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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.
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PMID:A case of diffuse plane normolipemic xanthomatosis associated with pancytopenia and monoclonal gammopathy. 1646 89

We present an original case report of a 58-year-old man with a history of histiocytoid Sweet and myelodysplastic syndrome who presented with a new onset of tender plaques on his oral commissures. A biopsy revealed a dense dermal neutrophilic infiltrate, leukocytoclasis without frank vasculitis, and, most notably, the presence of many xanthomatized cells. Clinical presentation and histologic features were most consistent with an acute neutrophilic dermatosis, which we believe to be a xanthomized variant of Sweet syndrome. Recent cases have described patients with similar clinical features and neutrophilic dermatosis in the setting of a normolipemic xanthomatosis. These cases were ultimately diagnosed as neutrophilic xanthoma, suggesting a possible histologic overlap between neutrophilic xanthoma and Sweet syndrome. Given these findings, we propose xanthomatized Sweet syndrome as a new histologic variant.
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PMID:Xanthomatized Neutrophilic Dermatosis in a Patient With Myelodysplastic Syndrome. 2775 96