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Multiple Firm, Painless Erythematous Papules With a Yellowish Hue—Diagnosis

Multiple Firm, Painless Erythematous Papules With a Yellowish Hue—Diagnosis Diagnosis: Generalized eruptive xanthomas associated with chylomicronemia syndrome and Frederickson type V hyperlipoproteinemia. Microscopic and laboratory findings and clinical course The punch biopsy specimen demonstrated a dermal infiltrate of foamy cells (lipid-filled macrophages) in small foci surrounded by lymphocytes, histiocytes, and neutrophils. On admission, the patient was treated with insulin and simvastatin for his hyperglycemia and hyperlipidemia. Subsequent laboratory workup (8 days after admission) after overnight fasting revealed an atherogenic lipoprotein profile (low levels of high-density lipoprotein cholesterol, relatively higher levels of low-density lipoprotein (LDL) cholesterol, low levels of apolipoprotein A-I, and high levels of apolipoprotein B); an abnormal electrophoretic lipid pattern (a low high-density lipoprotein cholesterol fraction and an elevated very-LDL [VLDL] cholesterol fraction [no intermediate-density lipoprotein cholesterol fraction could be detected]); hyperchylomicronemia; and a significantly elevated lipoprotein(a) level. Laboratory tests at that time revealed the following values: cholesterol, 525 mg/dL (13.60 mmol/L); triglycerides, 1230 mg/dL (13.90 mmol/L); and glucose, 126 to 220 mg/dL (7.0-12.2 mmol/L) (RRs as listed previously). During 2 months of follow-up, the patient’s skin lesions decreased in size and number, and no new lesions developed. Discussion Xanthomas represent the accumulation of lipid-rich macrophages known as foam cells. Eruptive xanthomas occur in a setting of elevated levels of plasma chylomicrons and hypertriglyceridemia, as may occur in uncontrolled diabetes mellitus. The use of isotretinoin, indomethacin, and the atypical antipsychotic agent olanzapine has also been reported to provoke the disease.1 The lipids in xanthomas are primarily free and esterified cholesterol, but occasionally other sterols and even triglycerides accumulate. This accumulation is usually the result of a high plasma concentration, with subsequent permeation of lipoproteins through the walls of dermal capillaries. The lipid is then taken up by dermal macrophages, which evolve into foam cells.2 The chylomicronemia syndrome is associated with marked hypertriglyceridemia (plasma triglyceride level, >2000 mg/dL [22.60 mmol/L) and such findings as abdominal pain, eruptive xanthomata, pancreatitis, mental confusion, memory loss, lipemia retinalis, and dyspnea.3 Insulin is required for the normal triglyceride-clearing function of lipoprotein lipase. Lipoprotein lipase hydrolyzes triglycerides that are present in circulating chylomicrons and VLDL to free fatty acids, which can be used as sources of energy or reesterified for storage. Whenever insulin deficiency is present, an acquired lipoprotein lipase deficiency exists, which results in impaired clearance of chylomicrons and VLDL, causing the resultant hypertriglyceridemia. Thus, chylomicronemia can occur in patients with uncontrolled diabetes who also have a familial form of hypertriglyceridemia (eg, Frederickson type I, IV, or V hyperlipoproteinemia).4,5 Type V hyperlipoproteinemia (mixed hypertriglyceridemia) is an extreme form, associated with extremely high levels of triglycerides and cholesterol, low levels of LDL, high VLDL fractions, and chylomicron positivity. Lipoprotein(a) lipoproteins are atherogenic, formed by the disulfide linkage of apolipoprotein(a) to apolipoprotein B-100 on an LDL.6 Xanthomas draw the clinician’s attention to the severe derangements of the metabolic state of the body, giving rise to potentially life-threatening pancreatitis or coronary artery disease. Article Submissions Clinicians, local and regional societies, and residents and fellows in dermatology are invited to submit quiz cases to this section. Cases should follow the established pattern and be submitted double-spaced. Photomicrographs and illustrations must be sharply focused with good color balance and submitted as separate JPG files with each file numbered with the manuscript number (eg, Fig_1DOF00000.jpg). Material must be accompanied by the required copyright transfer statement (see “Instructions for Authors”). Preliminary inquires regarding submissions for this feature may be submitted to Michael E. Ming, MD (offcenter.fold@uphs.upenn.edu). Manuscripts should be submitted electronically to archdermatol@jama-archives.org. Please indicate in your e-mail that the manuscript is a submission to Off-Center Fold. Reprints are not available from the authors. References 1. Chang HYRidky TWKimball ABHughes EOro AE Eruptive xanthomas associated with olanzapine use Arch Dermatol 2003;1391045- 1048PubMedGoogle ScholarCrossref 2. Naeyaert JM Xanthomas Ann Dermatol Venereol 2003;13065- 68PubMedGoogle Scholar 3. Chait ABrunzell JD Chylomicronemia syndrome Adv Intern Med 1992;37249- 273PubMedGoogle Scholar 4. Nayak KRDaly RG Images in clinical medicine: eruptive xanthomas associated with hypertriglyceridemia and new-onset diabetes mellitus N Engl J Med 2004;3501235PubMedGoogle ScholarCrossref 5. Hentges PPHuerter CJ Eruptive xanthomas and chest pain in the absence of coronary artery disease Cutis 2001;67299- 302PubMedGoogle Scholar 6. Luc GBard JMArvelier D et al. Lipoprotein (a) as a predictor of coronary artery heart disease: the PRIME Study Atherosclerosis 2002;163377- 384PubMedGoogle ScholarCrossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Multiple Firm, Painless Erythematous Papules With a Yellowish Hue—Diagnosis

Archives of Dermatology , Volume 141 (12) – Dec 1, 2005
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Multiple Firm, Painless Erythematous Papules With a Yellowish Hue—Diagnosis

Abstract

Diagnosis: Generalized eruptive xanthomas associated with chylomicronemia syndrome and Frederickson type V hyperlipoproteinemia. Microscopic and laboratory findings and clinical course The punch biopsy specimen demonstrated a dermal infiltrate of foamy cells (lipid-filled macrophages) in small foci surrounded by lymphocytes, histiocytes, and neutrophils. On admission, the patient was treated with insulin and simvastatin for his hyperglycemia and hyperlipidemia. Subsequent laboratory workup (8...
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References (6)

Publisher
American Medical Association
Copyright
Copyright © 2005 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.141.12.1595-h
Publisher site
See Article on Publisher Site

Abstract

Diagnosis: Generalized eruptive xanthomas associated with chylomicronemia syndrome and Frederickson type V hyperlipoproteinemia. Microscopic and laboratory findings and clinical course The punch biopsy specimen demonstrated a dermal infiltrate of foamy cells (lipid-filled macrophages) in small foci surrounded by lymphocytes, histiocytes, and neutrophils. On admission, the patient was treated with insulin and simvastatin for his hyperglycemia and hyperlipidemia. Subsequent laboratory workup (8 days after admission) after overnight fasting revealed an atherogenic lipoprotein profile (low levels of high-density lipoprotein cholesterol, relatively higher levels of low-density lipoprotein (LDL) cholesterol, low levels of apolipoprotein A-I, and high levels of apolipoprotein B); an abnormal electrophoretic lipid pattern (a low high-density lipoprotein cholesterol fraction and an elevated very-LDL [VLDL] cholesterol fraction [no intermediate-density lipoprotein cholesterol fraction could be detected]); hyperchylomicronemia; and a significantly elevated lipoprotein(a) level. Laboratory tests at that time revealed the following values: cholesterol, 525 mg/dL (13.60 mmol/L); triglycerides, 1230 mg/dL (13.90 mmol/L); and glucose, 126 to 220 mg/dL (7.0-12.2 mmol/L) (RRs as listed previously). During 2 months of follow-up, the patient’s skin lesions decreased in size and number, and no new lesions developed. Discussion Xanthomas represent the accumulation of lipid-rich macrophages known as foam cells. Eruptive xanthomas occur in a setting of elevated levels of plasma chylomicrons and hypertriglyceridemia, as may occur in uncontrolled diabetes mellitus. The use of isotretinoin, indomethacin, and the atypical antipsychotic agent olanzapine has also been reported to provoke the disease.1 The lipids in xanthomas are primarily free and esterified cholesterol, but occasionally other sterols and even triglycerides accumulate. This accumulation is usually the result of a high plasma concentration, with subsequent permeation of lipoproteins through the walls of dermal capillaries. The lipid is then taken up by dermal macrophages, which evolve into foam cells.2 The chylomicronemia syndrome is associated with marked hypertriglyceridemia (plasma triglyceride level, >2000 mg/dL [22.60 mmol/L) and such findings as abdominal pain, eruptive xanthomata, pancreatitis, mental confusion, memory loss, lipemia retinalis, and dyspnea.3 Insulin is required for the normal triglyceride-clearing function of lipoprotein lipase. Lipoprotein lipase hydrolyzes triglycerides that are present in circulating chylomicrons and VLDL to free fatty acids, which can be used as sources of energy or reesterified for storage. Whenever insulin deficiency is present, an acquired lipoprotein lipase deficiency exists, which results in impaired clearance of chylomicrons and VLDL, causing the resultant hypertriglyceridemia. Thus, chylomicronemia can occur in patients with uncontrolled diabetes who also have a familial form of hypertriglyceridemia (eg, Frederickson type I, IV, or V hyperlipoproteinemia).4,5 Type V hyperlipoproteinemia (mixed hypertriglyceridemia) is an extreme form, associated with extremely high levels of triglycerides and cholesterol, low levels of LDL, high VLDL fractions, and chylomicron positivity. Lipoprotein(a) lipoproteins are atherogenic, formed by the disulfide linkage of apolipoprotein(a) to apolipoprotein B-100 on an LDL.6 Xanthomas draw the clinician’s attention to the severe derangements of the metabolic state of the body, giving rise to potentially life-threatening pancreatitis or coronary artery disease. Article Submissions Clinicians, local and regional societies, and residents and fellows in dermatology are invited to submit quiz cases to this section. Cases should follow the established pattern and be submitted double-spaced. Photomicrographs and illustrations must be sharply focused with good color balance and submitted as separate JPG files with each file numbered with the manuscript number (eg, Fig_1DOF00000.jpg). Material must be accompanied by the required copyright transfer statement (see “Instructions for Authors”). Preliminary inquires regarding submissions for this feature may be submitted to Michael E. Ming, MD (offcenter.fold@uphs.upenn.edu). Manuscripts should be submitted electronically to archdermatol@jama-archives.org. Please indicate in your e-mail that the manuscript is a submission to Off-Center Fold. Reprints are not available from the authors. References 1. Chang HYRidky TWKimball ABHughes EOro AE Eruptive xanthomas associated with olanzapine use Arch Dermatol 2003;1391045- 1048PubMedGoogle ScholarCrossref 2. Naeyaert JM Xanthomas Ann Dermatol Venereol 2003;13065- 68PubMedGoogle Scholar 3. Chait ABrunzell JD Chylomicronemia syndrome Adv Intern Med 1992;37249- 273PubMedGoogle Scholar 4. Nayak KRDaly RG Images in clinical medicine: eruptive xanthomas associated with hypertriglyceridemia and new-onset diabetes mellitus N Engl J Med 2004;3501235PubMedGoogle ScholarCrossref 5. Hentges PPHuerter CJ Eruptive xanthomas and chest pain in the absence of coronary artery disease Cutis 2001;67299- 302PubMedGoogle Scholar 6. Luc GBard JMArvelier D et al. Lipoprotein (a) as a predictor of coronary artery heart disease: the PRIME Study Atherosclerosis 2002;163377- 384PubMedGoogle ScholarCrossref

Journal

Archives of Dermatology – American Medical Association

Published: Dec 1, 2005

Keywords: papule

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