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Research Letter |

Pathognomonic Palmar Crease Xanthomas of Apolipoprotein E2 Homozygosity-Familial Dysbetalipoproteinemia ONLINE FIRST

Matan Rothschild, MD1; Greg Duhon, MD1; Rashid Riaz, MD1; Vybhav Jetty, MD1; Naila Goldenberg, MD1; Charles J. Glueck, MD1; Ping Wang, PhD1
[+] Author Affiliations
1Cholesterol, Metabolism, and Thrombosis Center, MMA-Jewish Hospital, Cincinnati, Ohio
JAMA Dermatol. Published online September 07, 2016. doi:10.1001/jamadermatol.2016.2223
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This case series describes palmar crease xanthomas and other dermatologic manifestations of familial dysbetalipoproteinemia and mutant apoE2/2 genotype.

It is important for all clinicians, especially dermatologists, to recognize the rare diagnostic physical sign of familial dysbetalipoproteinemia (FD), palmar crease xanthoma (PCX),1 because it is an early warning sign to initiate diagnostic tests (apolipoprotein [apoE] genotyping, lipid profiling, documentation of dysbetalipoproteinemia by ultracentrifugation or nuclear magnetic resonance lipid profiling), and thus enables treatment. Familial dysbetalipoproteinemia, a rare familial hyperlipidemia, is very sensitive to treatment with fibrates and/or statins,2 with lipid normalization and reduction in risk of atherosclerotic cardiovascular disease (ASCVD).3 Approximately 20% of patients with FD have characteristic palmar xanthomas.1 Our specific aim was to assess dermatologic manifestations of FD (type 3 hyperlipoproteinemia) with the mutant apoE2/2 genotype, and emphasize the centrality of the dermatologist in a diagnostic-therapeutic role in identifying unique palmar xanthomas.

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Bilateral Palmar Planar Xanthoma

Numerous yellowish maculopapular lesions on the palms. Note the pathognomonic macular yellowish discoloration of the palms which is limited to the creases.

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