To the Editor.—
Alagille's syndrome, due to interlobular biliary duct deficiency, associates various cardiovascular, vertebral, ocular anomalies and especially, a typical facies, making the diagnosis possible at first glance, according to Alagille and coworkers.1 There are also cutaneous signs, however, that are rarely described in the literature. Therefore, it seemed worthwhile to report a case herein.
Report of a Case.—
When we saw this 22-year-old woman for the first time, we immediately noticed the facial aspect as described by Alagille and coworkers1: a rounded forehead, eyes deep in the sockets with subdued hypertelorism, a saddle nose, and a small chin (Fig 1). The patient complained of an unbearable pruritus with insomnia. Indeed, on the back side of her legs and ankles, there are discretely keratotic, multiple papules in streaks (Fig 2).Microscopic examination of the skin biopsy specimen disclosed deposits of an amorphous substance under a hyperkeratotic epidermis
Doutre M, Beylot C, Couzigou P, Beraud C. Lichen Amyloidosis in Alagille Syndrome. Arch Dermatol. 1991;127(10):1590-1591. doi:10.1001/archderm.1991.01680090156030