Abstract
We describe a dramatic case of class II non-Langerhans cell histiocytosis, xanthoma disseminatum, in a 30-year-old male patient with progressive involvement of the skin, vocal cords, eyes, bones and nerves in spite of chemotherapy with Vespesid and immunotherapy with interferon-gamma. At the age of 43 years, the patient required surgical clearance of airways, eyelids and peripheral nerves, but at present exhibits stable disease on a combination of lipid-lowering drugs including thiazolidinedione.
MeSH terms
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Adult
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Airway Obstruction / diagnosis
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Airway Obstruction / surgery
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Combined Modality Therapy
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Conjunctival Diseases / diagnosis
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Conjunctival Diseases / surgery
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Corneal Diseases / diagnosis
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Corneal Diseases / surgery
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Disease Progression
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Facial Dermatoses / diagnosis
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Facial Dermatoses / surgery
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Hand Deformities, Acquired / diagnosis*
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Hand Deformities, Acquired / surgery
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Histiocytosis, Non-Langerhans-Cell / diagnosis*
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Histiocytosis, Non-Langerhans-Cell / surgery
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Humans
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Hypolipidemic Agents / administration & dosage
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Male
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Otorhinolaryngologic Diseases / diagnosis
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Otorhinolaryngologic Diseases / surgery
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Palliative Care
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Skin Diseases / diagnosis
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Thiazolidinediones / administration & dosage
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Tracheotomy
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Ulnar Nerve Compression Syndromes / diagnosis*
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Ulnar Nerve Compression Syndromes / surgery
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Wrist Joint* / surgery
Substances
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Hypolipidemic Agents
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Thiazolidinediones
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2,4-thiazolidinedione