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September 1988

A Linear Fibrous Cord and Ulcer of the Lower Limb: A Limited Form of a Rare Dysmorphic Syndrome (Faciogenitopopliteal Syndrome)

Arch Dermatol. 1988;124(9):1443-1444. doi:10.1001/archderm.1988.01670090085025

To the Editor.—  In 1869 Trelat1 reported the first observation of a dysmorphic syndrome characterized by an alteration of the head, the lower limbs, and the genitourinary system; these features suggested the designation faciogenitopopliteal syndrome (FGPS).

Report of a Case.—  A small female (weight, 1870 g and length, 41 cm, both less than the third percentile, and head circumference, 31 cm, equal to the third percentile) was born at 39 weeks of gestation. She was the first child of a healthy mother; the father was normal clinically. Examination of the newborn disclosed a linear cutaneous defect on the posterior aspect of the right leg, extending from the heel to the ischial tuberosity. The lesion was composed of an inelastic fibrous cord limiting extension and abduction of the leg, and linear ulcers with focal epithelialization in the popliteal space (Fig 1). The homolateral labium majus was hypoplastic as was the