Background and Aims

Palmar striated xanthomas (PSX) are manifestations of dyslipidemias characterized by a yellow-to-orange coloration of palmar and finger creases. Their physiopathology is not well understood but they are frequently associated with sustained plasma accumulation of triglyceride (TG)-rich lipoprotein-remnants (IDL). PSX characterize type III dysbetalipoproteinemia, a remnant disease associated with apolipoprotein (apo) E resistance and increased peripheral artery disease (PAD) risk. Plasma IDL accumulation may however also occur in any condition recurrently interfering with lipoproteins hydrolysis or IDL clearance, including post-prandial dyslipidemia, partial lipoprotein lipase (LPL) deficiency or severe familial hypercholesterolemia (FH), including homozygous FH (HoFH).

Objective: To describe the characteristics of non-type III patients presenting PSX.

Methods

This study was performed among 636 non-type-III patients. Type III was defined by the presence of ≥3 criteria among: total cholesterol and TG approximately equal when reported in mg/dL, total cholesterol/apoB>6.2, TG/apoB<10, VLDL-Cholesterol/TG>0.5, apo E2/E2, tuberous or palmar xanthomas.

Results

A total of 25/636 (3.9%) of non-type III patients presented PSX, most (18/25) being women. FH was diagnosed in 16/25 (64%) individuals. All HoFH (n=8) had PSX, coronary artery disease and PAD. PSX also occurred in severe HeFH patients, in patients with partial LPL deficiency being apoE2 heterozygotes. Compared to patients without PSX, patients with PSX, FH or not, tended to be younger (p<0.01).

Conclusions

This study demonstrates that PSX may occur beyond type III. A significant proportion of non-type III patients with PSX are FH, and not exclusively HoFH. A better documentation of PSX would improve our understanding of their physiopathology and clinical significance.