Xanthoma disseminatum with respiratory tract involvement and fatal outcome.
نویسندگان
چکیده
Xanthoma disseminatum (XD) is a rare mucocutaneous xanthomatosis classified as a benign form of non-Langerhans' cell histiocytosis. The case history is presented of a 61 year old woman with XD who developed dyspnoea and spirometric features of airflow obstruction. Bronchoscopy and computed tomography confirmed involvement of the large and medium sized bronchi and she subsequently died from acute respiratory failure. The post-mortem findings and the importance of respiratory tract disease in this unusual condition are discussed.
منابع مشابه
A Case of Xanthoma Disseminatum Accentuating over the Eyelids.
Xanthoma disseminatum (XD) is a rare, benign non-familial mucocutaneous disorder, which is a subset of non-Langerhans cell histiocytosis. It is characterized by mucocutaneous xanthomas in a disseminated form typically involving the eyelids, trunk, face, and proximal extremities and occurs in flexures and folds such as axillae and the groin. Mucosal involvement of the respiratory or gastrointest...
متن کاملDisseminated intracranial xanthoma disseminatum: a rare case report and review of literature
BACKGROUND Xanthoma disseminatum (XD) is a rare benign histiocytic proliferating disease of non-Langerhans cell origin, which is clinically mainly characterized by cutaneous or mucous lesions. Although XD is acknowledged of one systematic disease, involvement of the central nervous system is quite rare. CASE PRESENTATION We presented one 34-year-old Chinese female with disseminated intracrani...
متن کاملXanthoma Disseminatum Presenting with Hoarseness
Introduction: Xanthoma disseminatum (XD) is a rare, benign, non-Langerhans cell histiocytic disorder with unknown etio-pathology. It manifests with multiple, grouped, red-brown to yellow papules and nodules involving the skin, mucous membranes, and internal organs with a predilection for flexures and the face. Case Report: We report a patient who presented with disseminated xanthomatous pa...
متن کاملXanthoma disseminatum: effective therapy with 2-chlorodeoxyadenosine in a case series.
BACKGROUND Xanthoma disseminatum is a rare nonfamilial disease characterized by lipid deposition in skin and internal organs due to histiocytic cell proliferation, classified as a benign non-Langerhans cell histiocytosis. This chronic disease has no known established treatment. We report 8 cases of xanthoma disseminatum with treatment outcomes in long-term follow-up and provide a review of the ...
متن کاملXanthoma disseminatum with extensive koebnerization associated with familial hypertriglyceridemia
FHTG: familial hypertriglyceridemia HDL: high-density lipoprotein LCH: Langerhan’s cell histiocytosis LDL: low-density lipoprotein VLDL: very low-density lipoprotein XD: xanthoma disseminatum INTRODUCTION Xanthoma disseminatum (XD) is a rare subtype of non-Langerhan’s cell histiocytosis (non-LCH). To date, just greater than 100 cases of XD have been reported; however, association with hyperlipi...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- Thorax
دوره 55 2 شماره
صفحات -
تاریخ انتشار 2000