Case Report: Eruptive Xanthoma in a 14-year-old boy Ryan M. Proctor, DO; Warren A. Peterson, DO; Michael W. Peterson, DO: Michael R. Proctor, DO; J. Ryan Jackson, DO; Andrew C. Duncanson OMS4; Allen E. Stout, DC. Dr. R. Proctor, Dr. W. Peterson, Dr. M. Peterson are from Aspen Dermatology Residency, Spanish Fork, UT; Dr. M. Proctor is from Arizona Desert Dermatology, Kingman, AZ; Dr. Jackson is from Sampson Regional Medical Center, Clinton, NC; OMS4 Duncanson is from Des Moines University, Des Moines, IA; DC Stout is from Stout Wellness Center, Fort Mohave, AZ. INTRODUCTION CLINICAL PHOTOS DISCUSSION Xanthomas are benign collections of lipid deposits that manifest as yellow to Eruptive xanthomas (EX) are often an indicator of severe red firm papules, nodules, or plaques. This yellow/red coloration is due to the hypertriglyceridemia and can indicate undiagnosed or Figure 1 Figure 2 carotene in lipids. Xanthomas are composed of lipid filled macrophages decompensated diabetes mellitus (DM).1 The triglyceride levels in known as “foam cells”. Xanthomas can be due to genetic disorders of lipid these patients can be >3000-4000 mg/dl (normal <150 mg/dl).2 metabolism, such as autosomal dominant hypocholesteremia or familial lipoprotein lipase(LPL) deficiency.1,2 They can also be caused by severe It is important to quickly diagnose and manage these patients due hypertriglyceridemia and have been associated with undiagnosed diabetic to the possible sequela of diabetes mellitus and hyperlipidemia. dyslipidemia, hypothyroidism, cholestasis, nephrotic syndrome, biliary Patients with eruptive xanthomas due to hypertriglyceridemia are atresia, and Alagille syndrome.4 Some cases have been reported in patients also at increased risk for acute pancreatitis.3 with alcohol dependence or medication use (e.g., isotretinoin).6 Most The diagnosis of eruptive xanthoma is often made easily based on xanthomas erupt in adulthood, however, xanthomas due to homozygous familial hypocholesteremia can present in childhood.2 the clinical picture although appropriate labs and biopsies may also There are multiple types of xanthomas including tuberous, tendinous, planar, be necessary. While this condition is typically seen in adults it may disseminated and eruptive. Eruptive xanthomas are 1-5 mm papules that often also manifest in children. develop rapidly and can present as a papular rash. They are most common on When it presents in the pediatric population the most common extensor surfaces as well as the buttocks and shoulders.7 Eruptive xanthomas finding is either uncontrolled or undiagnosed type-1 DM. have been noted to display the Koebner phenomenon - new lesions occurring 8 Incidence in this population is difficult to estimate, but a recent in areas of trauma. PUBMED search using the search terms eruptive xanthoma Biopsy of the xanthomatous papules will show infiltration of foam cells and multinucleate giant cells within the reticular dermis on hematoxylin-eosin revealed 180 articles while a subsequent search using the terms staining (Figure 3-5).2 In early lesions histiocytes are numerous and fully pediatric eruptive xanthoma incidence returned only 4 articles. developed foam cells are small in number. In an established papule, xanthoma cells have characteristic clear or foamy cytoplasm as the predominant cell type. They may also contain an admixture of lymphocytes and neutrophils.9 Eruptive xanthomas tend to exhibit smaller and fewer foam cells than other CASE REPORT types of xanthomas.2 They also tend to be predominantly triglyceride in the intra-cytoplasmic space while the other forms of xanthoma will have An obese, 14-year-old, Native American male, presented to his predominant amounts of cholesterol. The quantity of the lipids are also in a pediatrician complaining of a mildly itchy rash that started on his state of flux - this is thought to be associated with extracellular deposition - 9 upper extremities. Over the next 10 days it migrated onto his torso and this phenomenon is rare or absent in the other types of xanthomas. and legs. Parents admitted to starting a new laundry detergent but Laboratory analysis should be performed on all patients with a new diagnosis of eruptive xanthoma with unclear origin to evaluate for causes of denied being exposed to any other new products - including foods, hyperlipidemia. Labs looking for underlying metabolic disorders should medications, or lotions prior to the rash. include a fasting lipid panel and glucose level, HbA1c, CBC, thyroid The patient applied an OTC topical corticosteroid cream for hormones, as well as liver and renal function panels.1 several days without any improvement. During the review of Treatment of eruptive xanthomas is based on resolving the underlying systems poorly controlled diabetes mellitus (DM) and obesity were condition. A holistic approach involving diet modification, exercise and drug noted. The patient was otherwise well and thriving. Family history therapy is necessary to manage the hypertriglyceridemia and hyperglycemia. Eruptive xanthomas typically regress quickly with treatment of the lipid was remarkable for obesity, hyperlipidemia, and DM was present abnormality and underlying cause.10 on both sides of the family. On physical examination the patient had multiple crops of 1-3 mm HISTOLOGY non-umbilicated, yellow to red papules on his arms, legs, and torso REFERENCES (Figure 1). 1. Zak A, Zeman M, Slaby A, Vecka M. Xanthomas: clinical and Labs were ordered and serum triglyceride levels were 2,100 mg/dL 10x mag 40x mag pathophysiological relations. Biomed Pap Med Fac Univ Palacky Olomouc (normal <150 mg/dl) with serum cholesterol levels at 500 mg/dl Czech Repub. 2014;158(2):181-188. (normal <199 mg/dl). Fasting serum glucose levels were 250 mg/dl 2. Massengale W, Nesbitt L. Xanthomas. In:Bolognia J, Jorizzo J, Schaffer J. (normal 79-99) with a hemoglobin A1c of 13.4 (normal <5.7). Dermatology, Vol 2. Philadelphia, PA: WB Saunders Co; 2012: 1547-1556. Patient was referred to dermatology for consultation at which time 3. Makdsi F, Fall A. Acute pancreatitis with eruptive xanthomas. J Hosp Med. a punch biopsy was performed. Biopsy confirmed the diagnosis of 2010;5(2):115. EX and labs suggested it was most likely secondary to 4. Ravić-Nikolić A, Mladenović V, Mitrović S, Miličić V, Djukić A, Jovović- uncontrolled hyperlipidemia and diabetes mellitus. Dagović B, Savčić G. Generalized eruptive xanthomas associated with diabetic dyslipidemia. Eur J Dermatol. 2014;24(3):394-395. In a 2008 study of 47 pediatric patients the effects of Gemfibrozil 5. Smalley, CM, Goldberg, SJ. A pilot study in the efficacy and safety of was shown to be efficacious and well tolerated. The mean decrease Gemfibrozil in a pediatric population. J. Clin Lipodol. 2008 Apr;2(2):106-111. 5 in triglycerides was 47% while the mean HDL increased by 20%. 6. Cole, C. M. The best diagnosis is: Eruptive xanthoma. Cutis. Our patient was subsequently started on Gemfibrozil, referred to 2013;91(4):172, 177-178. endocrinology to manage his insulin regimen and follow up with a 7. Abdelghany M, Massoud S. Eruptive xanthoma. Cleveland Clinic Journal of diet and nutrition specialist. Follow up one week later already Medicine. 2015;82(4):209-10. showed improvement of the lesions as well as the pruritus. Within 8. Miwa N, Kanzaki T. The Koebner phenomenon in eruptive xanthoma. one month the lesions almost completely resolved and triglycerides Journal of Dermatology. 1992;19(1):,48-50. had returned to normal levels (Figure 2). 9. Mckee P, Calonje C, Granter S. Pathology of the skin with clinical correlations. Vol 1. Philadelphia, PA: Elsevier Limited; 2005: 542-543. 10. Parker F. Xanthomas and hyperlipidemias. J Am Acad Dermatol. 1985;13(1):1-30. RESEARCH POSTER PRESENTATION DESIGN © 2015 www.PosterPresentations.com.