Figure 2. Male patient with a benign prenatal form of hypophosphatasia. He had presented manifestations before birth but was only diagnosed with the disease at the age of 1 year and 8 months. He was started on asfotase alfa at the age of 2 years and 10 months. (A) Age 1 year and 2 months: upper limb radiograph showing diffuse bone hypomineralization, cupping, fraying, and widening metaphyses, thin cortical bone, and diaphyseal shortening. (B) Metaphyseal radiolucent “tongues” (arrows). (C) “Beaten copper” sign. (D) Age 5 years, after 2 years of treatment, showing improved bone mineralization and formation, cupping, fraying and widening metaphyses, and radiolucent “tongues.” The long bone became better defined, and the lytic and sclerotic metaphyseal areas resolved. Adapted from: Arch Endocrinol Metab. 2021;64(5):623-9.