Piezogenic papules in Ehlers-Danlos syndrome.

A 2-year-old girl was referred to our clinic for evaluation of Ehlers-Danlos syndrome. She had a history of easy bruising, and the physical examination showed hematomas, stretchy skin of velvety texture, and hyperflexible joints, suggestive of the presumed diagnosis. Her father had fullblown Ehlers-Danlos syndrome of the classic type featuring skin hyperextensibility, widened atrophic scars, and joint hypermobility. In both patients, piezogenic papules appeared on the medial and lateral aspects of the heels when standing upright with full weight (Figure). Piezogenic papules develop when high compressive stress causes herniation of subcutaneous fat into the dermis. The heels are a typical “locus minoris resistenciae.” Piezogenic papules are common in adults with an occupation that involves standing and in athletes such as marathon runners or weightlifters. However, in children and especially in toddlers, these papules are an unusual finding. Reports suggest an association of piezogenic papules and Ehlers-Danlos syndrome in which impaired collagen synthesis predisposes to various other kinds of herniation. EhlersDanlos syndrome summarizes a heterogeneous group of rare hereditary disorders with the common feature of connective