When encountered in children, xanthomas are most commonly associated with a group of disorders known as familial hyperlipidemias. Aside from cosmetic concerns, these xanthomas are typically asymptomatic, but when generalized pruritus is a prominent associated symptom, clinicians should consider a different set of diagnoses that includes cholestasis of the liver. In this article we present two illustrative cases of children with cholestatic disease, pruritus, and xanthomas and discuss other disorders that may include this triad.

A young girl presented for evaluation of hair loss. The mother stated that the patient was born with normal-appearing dark hair that fell out days after birth. This was replaced with "peach fuzz," with no subsequent regrowth of normal hair. She denied pruritus. Family history was significant for similar hair loss in the patient's maternal grandmother and maternal cousin. The patient had otherwise normal development. On physical examination, short, brittle terminal hairs of varying lengths covered the entire scalp. Follicular hyperkeratosis and perifollicular erythema were present, most prominently on the parietal and occipital scalp (Figure , A). Mild perifollicular keratotic papules were noted on the posterior upper arms. Teeth and nails showed no abnormalities. Her eyebrows and lashes were not affected. Results of trichography are shown (Figure , B).

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Onychomadesis occurs when the proximal nail plate becomes unattached to the underlying structures. When only one or a few nails are involved, the cause is usually physical trauma. A 23-year-old female presented with a 4-year history of recurrent onychomadesis involving her bilateral great toes that occurred during the springtime. While recurrent trauma from winter shoes or athletic activities may be a contributing factor, our patient was convinced that wearing tight-fitting socks during winter nights was the primary pathophysiologic process. By wearing socks only during the days and decreasing the amount of time the nails spent traumatized due to pressure, the patient's recurrent condition was eliminated.