A case of xanthoma disseminatum presenting as pedunculating nodules and plaques

Xanthoma disseminatum is a rare mucocutaneous xanthomatosis classified as a benign form of non-Langerhans cell histiocytosis. We describe an illustrative case with extensive mucocutaneous involvement which was unresponsive to treatment. The patient presented with numerous variable-sized, yellow-brown papules and confluent plaques on the perorbital, perioral, neck, axilla, upper chest, groin, perianal, antecubital fossae and popliteal fossae including the upper respiratory tract. She was treated with oral cyclophosphamide but showed no clinical improvement. She then developed dyspnea, dysphagia and experience defecation difficulties, so was treated with palliative surgery to help relieve the symptoms.