Cutaneous Normolipemic Plane Xanthoma with Supraglottic Involvement in a Patient with Hand-Schüller-Christian Disease

Abstract

Normolipemic plane xanthoma (NPX) is a histiocytic disorder characterized by yellow-orange plaques in the periorbital areas, neck, upper trunk, and flexural folds. Association with systemic disease or paraproteinemia has been reported previously, but rarely with Langerhans cell histiocytosis (LCH). We report a case of Hand-Schüller-Christian disease (a type of LCH) in a patient who developed NPX with supraglottic involvement. NPX developed after several courses of chemotherapy and the supraglottic xanthoma occurred about 2 years later. The coexistence of LCH and non-LCH histiocytic lesions in this patient could be a result of chemotherapy-induced changes or may be just coincidental.

Multiple Huge Tendinous Xanthomas with Normal Lipid Profiles in All Extremities

“…Xanthomas are composed of cholesterol-rich materials and can occur anywhere. They usually accompany familial hypercholesterolemia, though some case reports have described xanthoma in patients with a normal plasma lipid profile [5,6]. Xanthomas usually occur in association with another diseases or syndromes such as CTX, beta sitosterolemia, or overproduction of apolipoprotein B [6][7][8].…”

“…A few case reports have been issued on xanthomas with a normal lipid profile, most concerned plane xanthoma [5] and others tendinous xanthoma of Achilles tendon [6]. Here, we report a rare case of huge multiple tendinous xanthomas in all extremities with a normal lipid profile that were large enough to cause pain, motor disturbance, and poor cosmesis.…”

“…Xanthomas are grayish-yellow masses composed of lipid-filled foamy histiocytes, extracellular cholesterol, giant cells, and inflammatory cells [1], and are usually caused by cholesterol accumulation in familial hypercholesterolemia or some other diseases and may be the mainstay of the clinical diagnoses of these lipid disorders [2]. Xanthomas usually involve skin and subcutaneous tissue, and sometimes involve tendon and bone deeply [3][4][5]. Tendinous xanthoma usually involves hand extensor tendons, but may also be found on elbow, patella, and Achilles tendons.…”

Solitary Polypoid Laryngeal Xanthoma

“…Noncutaneous xanthomas are infrequent and exceptional in the larynx, with only eight cases reported [6]. Some of these cases involve disseminated xanthomas in the larynx [7].…”