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Unusual and memorable

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    A 29 year old women presented with painful stiff fingers on her left hand. The pain was associated with intermittent swelling and limitation of motion of her digits. She was otherwise well with no history of psoriasis or other joint problems. On examination there was diffuse bony swelling of the second and third fingers of her left hand with a reduced range of movement. The overlying skin was shiny and erythematous (fig 1). Radiographs of the hands revealed endosteal hyperostosis of the second and third metacarpals (fig 2 ). Scintigraphy showed an increased accumulation of radionuclide in these areas (fig 3).

     Melorheostosis is a rare bone disorder.1It usually presents with joint pain, intermittent swelling, and muscle contractures below the age of 20 years but may occur as late as the fourth or fifth decades. The disease is usually limited to a single limb, in which one or more bones may be affected. The lower limbs are more commonly affected than the upper limbs. Radiographic appearances are highly characteristic. Osseous excresences extend along the length of the bone and may be associated with endosteal hyperostosis partially or completely obliterating the medullary cavity. Soft tissue calcification and ossification occurs frequently. The cause of melorheostosis is unknown although investigations have indicated possible associations with neurofibromatosis, tuberous sclerosis, hypophosphataemic rickets, and haemangiomas.



    • Contributors:a pendleton, gd wright, sd roberts, Royal Victoria Hospital, Belfast.