A 21-yr-old man was being seen for pain and weakness in his right fingers, hand, and forearm for the past 3 mos. Upon detailed questioning, he related that his symptoms ensued after excessive exercise (heavy lift, push-up, pull-up, rope climb, etc) but not during routine daily activities or during rest. The medical history was otherwise noncontributory. Upon physical examination, it was found that there was hyperesthesia/algesia on the dorsal side of the fourth and fifth fingers and the forearm on the right side. Secondary to pain, wrist flexion was limited.
Radiologic evaluations of the hand and the forearm revealed extensive sclerosis and were consistent with a diagnosis of melorheosteosis (Fig. 1 A, B). An increased activity in the mentioned areas was also observed in radionuclide triphasic bone scintigraphy. The authors prescribed rest and nonsteroidal anti-inflammatory drugs, and the patient’s condition improved significantly. After 10 mos of follow-up, despite the persistence of radiologic findings, the patient was free of any painful complaints.
Melorheostosis was originally described by Leri and Joanny1 in 1922. It is a rare disorder of unknown etiology characterized by dense sclerotic bone. X-rays feature a “flowing” hyperostosis of the cortex, which resembles wax dripping down one side of a candle.2 This appearance also gave the anomaly its name, derived from the Greek words for member (melos) and flow (rhein).3 Soft-tissue abnormalities are common, and skin lesions resemble scleroderma. Joint swelling, muscle contractures, and tendon and ligament shortening, causing pain and limited range of motion, can be encountered.4 This condition may involve any bone in the body; however, the hand is a relatively uncommon site of presentation.
Radiographic findings have been the cornerstone of the diagnosis. There is usually a distinct demarcation between the affected and the normal bone.4 Routine laboratory findings are usually normal. Bone scintigraphy has invariably positive findings, revealing an increased uptake of tracer predominantly in the cortex.
The treatment of melorheostosis is suggested to be individualized based on the patient’s lifestyle, pain level, progression of disease, and age. The authors suggest that the diagnosis of melorheostosis be kept in mind in cases of hyperostosis of bones.
1. Leri A, Joanny J: Une affection non décrite des os hyperostose “en coulée” sur toute la longeur d’un member ou “melorhéostose.” Bull Mem Soc Med Hosp 1922; 46: 1141–5
2. Kurklu M, Ozkan H, Komurcu M, et al.: Melorheostosis in the foot. Am J Phys Med Rehabil 2007; 86: 868
3. Greenspan A, Azoua EM: Bone dysplasia series. Melorheostosis: Review and update. Can Assoc Radiol J 199I; 50: 324–30
4. Waddell C, Demos TC, Lomasney L, et al.: The case: Your diagnosis? Orthopedics 2005; 28: 720–5