Eruptive xanthomas
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https://doi.org/10.5070/D30dp3p2gqMain Content
Eruptive xanthomas
Joseph F Merola MD, Stephanie J Mengden MD, Anthony Soldano MD, Karla Rosenman MD
Dermatology Online Journal 14 (5): 10
Department of Dermatology, New York UniversityAbstract
A 26-year-old woman presented for evaluation of multiple, red-yellow papules that had been appearing over the course of 2 months on the extensor surfaces of the upper extremities, dorsal aspects of the hands, thighs, and buttocks. Serum lipid evaluation showed hypertriglyceridemia in a patient with a recent history of pancreatitis. A diagnosis of eruptive xanthomas was made based on clinical, laboratory, and histopathologic findings.
Clinical synopsis
A 26-year-old-woman presented to the Dermatology Clinic of the Bellevue Hospital Center in May, 2007, for evaluation and treatment of multiple, red-to-yellow papules that had been increasing in number for the past 2 months. The lesions began on the hands, with subsequent spread to the forearms and later to the thighs and buttocks. The Patient denied pain or pruritus at the site of the eruption. Medical history included pancreatitis with a pseudocyst and deep venous thrombosis. The patient denied a family history of similar lesions. She had recently been started on gemfibrozil for serum lipid abnormalities.
Physical examination
Multiple, red-to-yellow papules were present over the extensor aspects of the arms, dorsal aspects of the hands, thighs, and buttocks. No lesions were noted over the cornea, eyelids, mucous membranes, palms, or achilles tendons. A single, yellow-red, linear plaque was noted on the extensor aspect of the right forearm.
Figure 1 | Figure 2 |
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Laboratory data
A complete blood count and chemistry profile were normal. The blood glucose was 295 mg/dl. Total cholesterol was 308 mg/dl, high-density lipoproteins 18 mg/dl, and triglycerides 2398 mg/dl.
Histopathology
In the papillary and upper reticular dermis, there is a nodular infiltrate of histiocytes with abundant, finely vacuolated cytoplasm that is indicative of lipidized macrophages, so called foam cells. Extracellular deposits of lipid are also seen. There is an accompanying mild perivascular, lymphocytic infiltrate.
Comment
Eruptive xanthomas appear clinically as small, erythematous-to-yellow papules that are distributed over the hands, extensor surfaces, and buttocks. Early lesions may have an erythematous halo and are associated with pruritus and tenderness [1]. A Koebner reaction may occur [2]. Histopathologically, foamy macrophages are present in the dermis with an inflammatory infiltrate of lymphocytes and neutrophils. The eruption is associated with both primary and secondary hypertriglyceridemia, with primary hypertriglyceridemia observed in type I (elevated chylomicrons), type IV (elevated VLDLs), and type V (elevated chylomicrons and VLDLs) dyslipidemias. Underlying the abnormality can be a deficient activity of lipoprotein lipase, dysfunctional apoprotein C-II, impaired insulin activity, or hepatic overproduction (as seen in endogenous familial hypertriglyceridemia) [3, 4, 5]. External factors, such as the introduction of oral retinoid therapy, development of diabetes mellitus, and alcohol abuse, may worsen hypertriglyceridemia in these patients and may trigger or worsen skin lesions [3, 5]. Rarely, eruptive xanthomas have been reported with hypothyroidism, nephrotic syndrome, and von Gierke glycogen storage disease [6]. Treatment involves identifying the underlying cause of the hypertriglyceridemia and commencing dietary and pharmacologic interventions to lower circulating levels.
References
1. Massengale WT, Nesbitt Jr LT. Xanthomas. In: Bolognia JL, et al, eds. Dermatology. New York: Mosby, 2003: 14482. Miller DM, Brodell RT. Eruptive xanthomatosis with linear koebnerization. J Am Acad Dermatol 1995; 33:834
3. Breckenridge WC, et al. Apolipoprotein and lipoprotein concentrations in familial apolipoprotein C-II deficiency. Atherosclerosis 1982; 44:223
4. Hentges PP, Huerter CJ. Eruptive xanthomas and chest pain in the absence of coronary artery disease. Cutis 2001; 67:299
5. Santamarina-Fojo S. The familial chylomicronemia syndrome. Endoctinol Metab Clin North Am 1998; 27:551
6. Parker F. Xanthomas and hyperlipidemias. J Am Acad Dermatol 1985; 13:1
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