Eruptive Xanthoma

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Clinical: Histology:

IHC repertoire: Staging: Prognosis: Adverse variables: Treatment:

Diabetic xanthoma, xanthoma diabetacorum Serum hypertriglyceridemia and/or elevated VLDL Diabetes mellitus, oral estrogens, acute ethanol ingestion, lipoprotein lipase deficiency, Type IV/V hyperlipoproteinemia, pregnancy, nephrotic syndrome, hypothyroidism, intravenous miconazole, oral 13-a's-retinoic acid

Crops of red-yellow papules on buttocks and thighs

Normal epithelium, early-perivascular lipid and neutrophils, later perivascular histiocytes and foam cells seen


5% mortality

Acute pancreatitis, serum triglycerides >2000 mg/dL Dietary modification, weight loss

Eruptive xanthoma (EX) is a serious systemic dyslipid-emia with distinctive cutaneous features. Although the dermatologic manifestations are not in themselves serious, their presence may be the harbinger of serious visceral disease. EX is an uncommon disease with a near equal gender incidence, principally seen in two age groups with different predisposing factors. Among children and young adults genetic disturbances in lipid metabolism are largely responsible and include lipoprotein lipase deficiency and Type I and V hyperlipoproteinemia (1). In older adults, acute ethanol ingestion and endocrinologic disturbances including hypothyroidism and diabetes mellitus are often observed. Common to these clinical settings is the presence of serum hypertriglyceridemia and/or elevated very low-density lipoproteins (VLDL) (2). Triglycerides are transported in the serum as a composite, known as a chylomicron, consisting of lipid with the apolipoproteins B-48, C-II, C-III, E, A-I, and A-IV. The apolipoproteins are critical in the metabolism of the chylomicrons. Chylomicrons are synthesized in the intestine and circulate in the serum, passing off triglycerides to the peripheral tissue endothelial capillaries via the enzymatic action of lipoprotein lipase and the binding of apolipoprotein C-II. Chylomicron remnants return to the liver via apolipoprotein E receptors for metabolic breakdown. VLDL particles originate within the liver, contain relatively more cholesterol than triglyceride, yet perform a similar function, delivering triglycerides to the peripheral tissues and returning to the liver for breakdown. These lipids are usually markedly elevated in the serum as a directly inherited consequence of faulty lipid metabolism such as lipoprotein lipase or apolipoprotein C-II deficiency or as an indirect mechanism following heavy ethanol consumption (3). As the liver is critical to the metabolism of lipoproteins, ethanol and various medications including miconazole and retinoic acid that are similarly metabolized within or are affected by the status of the liver may result in lipid abnormalities (4,5). Similarly, serious endocrinologic disturbances, such as hypo-thyroidism or diabetes mellitus, or intrinsic liver disorders, such as the condition of fatty liver of pregnancy, may affect the metabolism of liver lipoprotein synthesis (6). Combined elevation of chylomicrons and VLDL constitutes Type 5 hyperlipoproteinemia, the most common hereditary-mediated cause of EX (7). This disorder is aggravated by secondary acquired factors such as obesity, which is known to be associated with elevated VLDL levels and insulin resistance. Insulin resistance and diabetes mellitus are also associated with defective lipolysis of chylomicrons and VLDL (8,9). The pathogenesis of the skin lesions and associated visceral findings relate to the presence of intravascular lipid as well as the escape of lipids from the circulation, evoked inflammation, and resulting foam cell formation. Marked elevation in intravascular lipids may induce platelet clumping and vascular plugging as observed in the retinal artery, and the complications of lipemia retinalis, capable of inducing blindness. Extravascular lipids may incite acute inflammation that, in conjunction with the lipid itself, produces free radical and oxidant-mediated cell membrane destruction. These mechanisms underlie the formation of the typical cutaneous lesions and the development of the most important systemic complication of acute pancreatitis. The risk for acute pancreatitis rises precipitously when triglyceride levels exceed 2000 mg/dL. In time, through a less-well understood mechanism, the extravas-cular lipid is scavenged by histiocytes forming foam cells.

EX presents as 1- to 4-mm reddish-yellow papules on the buttocks or extensor surfaces of the thighs and arms (Figure 31.1) (3). The lesions may be surrounded by an erythematous halo and usually occur in crops that may coalesce, forming plaques. Their presence is indicative of a triglyceride level that typically exceeds 2000 mg/dL. Other clinical stigmata of EX include ocular, abdominal, and pulmonary findings. The most important ophthalmic complication is lipemia retinalis. On fun-doscopic examination, the retinal arteries and veins appear white and engorged. The risk of lipemia retinalis increases when serum triglyceride levels exceed 4000 mg/ dL. Abdominal pain is a common accompaniment to EX. The source of the pain may be due to acute pancreatitis or hepatosplenomegaly. Chest pain or dyspnea may also occur due to decreased pulmonary oxygen diffusing capacity that may be aggravated by abnormal hemoglobin oxygen affinity. The natural history of the cutaneous lesions is gradual resolution with successful treatment within 6 to 8 weeks of instituting therapy.

The histologic changes of EX are often subtle, particularly in early lesions (10). The changes principally involve the dermal capillaries and perivascular tissue and consist of initial accumulations of neutrophils that in time are replaced by lymphocytes and histiocytes, including foam cells (Figures 31.2-31.4). Extravascular lipid or fibrin may be identified as a granular precipitate seen within the vicinity of the blood vessels.

The prognosis of EX is generally favorable with prompt recognition and institution of therapy (2,3). Delayed diagnosis, marked elevation of serum triglycerides, or acute

Eruptive Xanthomas And Lipemia Retinalis
Figure 31.1. Orange-yellow grouped papules on buttocks of patient with eruptive xanthoma.

pancreatitis is associated with a worse prognosis. In its initial setting, acute pancreatitis constitutes the most important cause of mortality with an overall rate of 5%. Important complications additionally include lipemia retinalis and hepatosplenomegaly.

Therapy should be aimed at measures that reduce serum levels of triglyceride and/or VLDL and that palliate complications of EX or its disease associations such as diabetes mellitus, obesity, or ethanolism (2,3). Long-term dietary fat restriction, weight loss, and appropriate glucose-lowering measures aimed at maintaining serum triglyceride levels below 1500 mg/dL should be instituted.

Figure 31.2. Dome-shaped papule with dermal inflammatory infiltrate.
Eruptive Xanthomas And Lipemia Retinalis
Figure 31.3. Medium power photomicrograph depicting perivascular and interstitial inflammatory infiltrate.

Figure 31.4. High power detail with perivascular foamy histiocytes and neutrophils.

Figure 31.4. High power detail with perivascular foamy histiocytes and neutrophils.

Xanthelasma Pictures

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