Verrucous plane xanthomas secondary to lipoprotein X dyslipidemia in the context of cholestatic fulminant hepatitis: A case report

Abstract

Cutaneous xanthomas are the result of dermal deposition of lipid, mostly caused by disorders of lipid metabolism. Less commonly, they occur in the setting of cholestatic liver disease, leading to accumulation of lipoprotein X, a rare form of dyslipidemia that does not respond well to conventional treatments. We describe an atypical presentation of sudden diffuse xanthomas secondary to lipoprotein X dyslipidemia in the context of cholestatic fulminant hepatitis caused by trimethoprim-sulfamethoxazole hypersensitivity. Histopathology was also atypical and showed an unusual verrucous appearance consisting of overlying epidermal hyperplasia with hyperkeratosis. Our patient had significant improvement, after normalization of her lipid panel under cholestyramine and 13 sessions of apheresis, with topical corticosteroids offering some relief. This rare case shows the importance of recognizing atypical presentations of xanthomas, particularly when they do not respond to conventional dyslipidemia treatments.

Diffuse xanthomas in a patient with lipoprotein X hyperlipidemia

“…We have summarized these cases in Table I . 3 , 4 , 7 , 8 , 9 Three of the 6 reported patients with cutaneous xanthomas and LpX hyperlipidemia had primary biliary cholangitis, whereas 2 had PSC. All 6 reported cases were women, with ages ranging from 28 to 51 years.…”

“… 8 Of the 3 patients treated with plasmapheresis to manage their LpX-related lipid abnormalities, all 3 experienced improvement or complete regression of their xanthomas. 3 , 7 , 9 …”

Use of therapeutic plasma exchange to remove lipoprotein X in a patient with vanishing bile duct syndrome presenting with cholestasis, pseudohyponatremia, and hypercholesterolemia: A case report and review of literature

“…[1][2][3] Lp-X can be observed in up to 45% of patients with liver dysfunction and cholestasis. 1,2,[4][5][6][7][8][9][10][11][12][13][14][15] Although rare, it can be also seen in pregnancy, 16 graft vs host disease, 15,17 or lecithincholesterol acyltransferase deficiency. [18][19][20] Lp-X is produced when extra-and/or intra-hepatic cholestasis occurs, causing bile lipoprotein to reflux into the circulation and bind to albumin to form Lp-X.…”

“…Lipoprotein X (Lp‐X) is an abnormal lipoprotein which is a spherical particle 50 to 70 nm in diameter with a high content of phospholipid (66% by weight) and unesterified cholesterol (22% by weight) 1‐3 . Lp‐X can be observed in up to 45% of patients with liver dysfunction and cholestasis 1,2,4‐15 . Although rare, it can be also seen in pregnancy, 16 graft vs host disease, 15,17 or lecithin‐cholesterol acyltransferase deficiency 18‐20 .…”