Painful Piezogenic Pedal Papules on a Child with Ehlers-Danlos Syndrome

Abstract

A 5-year-old girl with Ehlers-Danlos syndrome developed painful piezogenic pedal papules. She had suffered from pain in her heels for the previous two years and had undergone extensive orthopedic examinations that revealed no abnormalities. On admission, she had typical signs associated with piezogenic pedal papules (PPP), with pain induced by standing and disappearance of the lesions and the pain on relief of pressure. These papules were due to herniation of subcutaneous fat into the dermis, possibly because of structural defects of the connective tissue. To the best of our knowledge, this is the first reported case of painful PPP occurring in childhood. We suggest that the herniation of subcutaneous fat in our patient was most probably due to the connective tissue defect that occurs in the Ehlers-Danlos syndrome.