CASE
A healthy 67-year-old Black man presented with flat, skin-colored papules and plaques on his eyelids and epicanthic folds (Figure 1). The lesions were only present around the eyes, had a slight yellow hue, and were asymptomatic.
MULTIPLE-CHOICE QUESTION
Sebaceous hyperplasia
Syringomas
Xanthelasma palpebrarum
Necrobiotic xanthogranuloma
Cutaneous Erdheim-Chester disease
ANSWER
Answer: C. Xanthelasma palpebrarum.
DISCUSSION
Xanthelasmata, localized collections of lipid deposits, classically present as yellow-hued papules and plaques. They are typically found near the eyelids and canthal folds, without cutaneous xanthomas present elsewhere on the body (Nair & Singhal, 2017). Notably, the color is harder to discern in patients with darker skin tones as they can appear skin colored with only a hint of yellow compared with the classic yellow plaques seen in patients with lighter skin types.
The prevalence of xanthelasmata in the United States is 0.6% in men and 1.0% in women (Bergman, 1994). Diagnosis of xanthelasmata should prompt the measurement of blood lipid levels, including cholesterol and triglycerides. Dyslipidemia is very common; one study showed 41% of patients had elevated blood lipids, and 94% had decreased high density lipoproteins (Bates & Warren, 1989). If diagnosed before the age of 40 years, patients should be screened to rule out inherited disorders of lipoprotein metabolism, such as Types II and IV familial hyperlipidemias. Patients with familial hyperlipidemias may also have lipid deposits present around the cornea, known as arcus corneae, as well as xanthomas present in tendons and on extensor surfaces (Bates & Warren, 1989). Additional causes for xanthelasmata include hypothyroidism, diabetes, pancreatitis, renal disease, and cirrhosis as well as cyclosporine, retinoids, and glucocorticoids (Rohrich et al., 2002).
Sebaceous hyperplasias are benign enlargements of otherwise normal sebaceous glands, typically found in sebum-rich areas such as the face. They classically present as skin-colored or yellow papules with central umbilication.
Syringomas are benign sweat gland tumors, typically presenting as small, round, translucent papules around the eyes and eyelids. They typically appear after puberty, most commonly in middle-aged women.
Necrobiotic xanthogranuloma (NXG) is a rare disorder that can appear with yellow papules coalescing into plaques or nodules. These lesions often have associated itching, burning, pain, or surface changes such as ulceration, lid swelling, or associated ocular field defects. Most of these lesions appear on the trunk or extremities and subsequently involve the periocular area, which is the most common site of involvement. NXG skin lesions often appear in the sixth decade of life and have a chronic, indolent, and progressive course (Girisha et al., 2012). Monoclonal gammopathies are found in 80%-90% of NXG cases (Girisha et al., 2012).
Erdheim-Chester disease is associated with an excessive production and accumulation of histiocytes. Cutaneous lesions, occurring in 25% of these patients, appear as papules and plaques around the eyelids that can mimic xanthelasmata. Erdheim-Chester is a multisystem disease with bone pain and constitutional symptoms such as fever, chills, night sweats, and unintentional weight loss (Kobic et al., 2020).
Xanthelasmata are usually only of cosmetic concern as they are often asymptomatic. Common treatments include surgical excision, application of chlorinated acetic acids such as bichloroacetic acid, and electrocautery. Regardless of the mode of treatment, recurrence is common, especially in those patients with hereditary forms of high cholesterol. After primary surgical excision, the recurrence rate was reported to be 40% (Mendelson & Masson, 1976). In a study analyzing the efficacy of topical 100% bichloracetic acid, 85% of patients experienced initial complete clearing and 72% of their lesions did not require retreatment over an average period of 68 months. Eighty-three percent of the poorly responsive or recurrent lesions after application of the bichloracetic acid were associated with high cholesterol (Haygood et al., 1998). There is evidence that correcting dyslipidemia with lifestyle modifications such as regular physical exercise, low-fat diet, and certain medications can lead to slow resolution in some patients (Rohrich et al., 2002).
Like xanthelasmata, sebaceous gland hyperplasia and syringomas are benign lesions that are treated only for cosmetic purposes. Treatment of NXG is focused on the underlying hematologic malignancy.
REFERENCES