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Melorheostosis a rare bone disorder whose initial manifestations include swelling of joints, pain, and limitation of motion. Eventually profound muscle contractures, tendon and ligament shortening, and soft tissue involvement with severe growth disturbances may ensue. Scoliosis, joint contracture and foot deformities may be seen. Frequently the radiographic alterations are limited to a single limb, more often the lower extremity. Cortical hyperostosis having the appearance of bone excrescences extending along its length resembles candle wax flowing down the side of a lit candle (Fig.1). This hyperostosis may reach the carpus and tarsus as well as the metacarpals, metatarsals or phalanges. In the carpal and tarsal areas, more discrete round foci may resemble the findings of osteopoikilosis, whereas in the flat bones, radiating sclerotic patches are seen. In addition, soft tissue calcification and ossification may result in complete ankylosis of the joint. Scintigraphy reveals areas of increased skeletal accumulation of radionuclide, and MR imaging is also of value in diagnosis. On MR images, bone and soft tissue lesions are of low signal intensity on all pulse sequences. (more)

The patient is a 4 year old girl with a history of an inversion injury to the right ankle.

J Bone Joint Surg Br Fairbank 30-B (3): 533. 1948
From an Atlas of General Affections of the Skeleton

The etiology and incidence of melorheostosis is unknown. It is often discovered as an incidental finding on radiographs acquired for other reasons. Other patients may have bone pain, limited range of motion, and joint fusion. The disease consists of cortical hyperostosis ("candle wax") in a dermatomal distribution. The location is usually diaphyseal. The process often crosses joints and may result in flexion contractures. The overlying skin may become thickened.

The Melorheostosis Association is a not-for-profit organization dedicated to finding the cause, treatments and cure for melorheostosis. Our focus is on promoting greater awareness and understanding of this progressive disease and its manifestations through education, research, communication and advocacy efforts on behalf of those affected by it as well as those dedicated to alleviating it. Patient Provider

A warm welcome to the web site of the UK Melorheostosis Association; a not-for-profit organisation dedicated to finding the cause, treatments and cure for melorheostosis and associated rare bone diseases. We have 3 core objectives: 1 To locate every British and European sufferer of melorheostosis, and encourage them to work with us by sharing details of their circumstances and condition 2 To support our friends at the US Melorheostosis Association (www.melorheostosis.com) in their bid to help unify the worlds of medicine and science in order to find a cure quickly 3 To provide a virtual meeting point where sufferers and their families can exchange information and provide mutual support Patient Provider

Meloreostosis, Leri's disease, flowing periosteal hyperostosis This disease was described by Leri and Joanny in 1922. It is a rare, non-hereditary lesion that affects both sexes. It is usually apparent in early childhood and even in the first few days of life. About 50% of persons affected will develop the symptoms by 20 years of age. The classic radiographic appearance is that of sclerotic lesions of bones that look like wax dripping down the side of a candle. the name is derived from the greek melos "limb" and rhein "to flow".

"A form of osteosclerosis or hyperostosis extending in a linear track through one of the long bones of an extremity, and consisting of proliferated ivory-like new bone. "Melo-" is from the Greek, limb and "-rheostosis" refers to streaks (rheo- from the Greek, flow), in the bones. (Dorland, 27th ed)" 3 synonyms or equivalents were found. Melorheostosis aka/or Candle bones disease aka/or Candle wax disease may cause or feature Symptoms and Signs Hemihypertrophy X-ray abnormalities Osteosclerosis

Melorheostosis is an uncommon bone disorder which often presents in children or young adults as extremity pain with limited range of motion. The symptoms may be progressive. In general, children are more severely affected and may develop muscle contractures and ligament and tendon shortening. It may be incidentally discovered in asymptomatic adults. Associated disorders have been reported; these include overlying linear scleroderma, osteopoikilosis, osteopathia striata, neurofibromatosis, tuberous sclerosis, vascular lesions, and hypophosphatemic rickets.

General Discussion Melorheostosis is a rare and progressive disease characterized by thickening (hyperostosis) of the outer layers of bone (cortical bone). Melorheostosis affects both bone and soft tissue growth and development. While the disorder is benign, it often results in severe functional limitation; extensive pain; malformed or immobilized muscles, tendons or ligaments; and limb, hand or foot deformity.

A 47-year-old male presented with a history of left foot pain, discomfort, difficulty standing and walking, and a sensation of “walking on marbles” for several months; he also was unable to fit his foot into his shoe. The patient’s past medical history was significant for left foot sesamoiditis and remote surgical procedures on the left foot. A plain radiograph of the left foot showed mixed sclerosing dystrophy in addition to a “candle-wax-like” appearance and thickening of the cortices of the calcaneum, third through fifth metatarsals, and proximal phalanges. An MRI scan showed signal alteration and hypointensity on both T1- and T2-weighted sequences. A three-phase bone scan with Tc-99m-MDP scintigraphy demonstrated moderately intense uptake of the radiopharmaceutical localized to the left lateral foot, which corresponded to the sclerosing lesion seen on the plain radiographs and MRI. (more)

CLINICAL FEATURES This disorder is similar to Ollier disease, Albright polyostotic fibrous dysplasia, Sturge-Weber syndrome, Klippel-Trenaunay-Weber syndrome, and others in which no mendelian basis has been established. The designation combines roots meaning limb, flow, and bone. The bones of the limbs (but not limited to these) show asymmetrical bands of sclerosis in a flowing pattern. The changes may extend across joints. The soft tissues overlying the bones are also sclerotic.

"...a rare bone disorder (of unknown etiology) whose initial manifestations include swelling of joints, pain, and limitation of motion. Eventually profound muscle contractures, tendon and ligament shortening, and soft tissue involvement with severe growth disturbances may ensue. Scoliosis, joint contracture and foot deformities may be seen." Encyclopaedia of Medical Imaging Volume III:1 Melorheostosis usually presents in a sclerotomal distribution. Early clinical findings include joint swelling, limitation of motion and pain. Contractures, ligamentous and tendinous shortening and even soft tissue involvement may ensue.(1) Radiographic findings are often limited to a single limb where more than one bone is often involved. The lower extremity is more often affected than the upper extremity. The axial skeleton is often spared. The classic finding is that of "peripherally located (cortical) hyperostosis...in one bone or a series of bones. The appearance of the osseous excrescences extending along the length of the bone simulates that of candle wax flowing down the side of the lit candle" Resnick: Bone and Joint Imaging, Second Edition. W.B Saunders Company 1996. The appearance of the lesion(s) gave the anomaly its name, which is "taken from the Greek words for member (melos) and flow (rhein). There is usually a distinct demarcation between the affected and normal bone. Dense, sclerotic linear areas are seen mainly in the cortex but also extending into the cancellous bone. It may co-exist with osteopoikilosis and osteopathia striata as well as with tumours or malformations of blood vessels or lymphatics. Soft-tissue ossifications at the site of the joint are common." University of Washington, UWMC Roosevelt Clinic, Musculoskeletal Radiology

Definition Melorheostosis is "...a rare bone disorder (of unknown etiology) whose initial manifestations include swelling of joints, pain, and limitation of motion. Eventually profound muscle contractures, tendon and ligament shortening, and soft tissue involvement with severe growth disturbances may ensue. Scoliosis, joint contracture and foot deformities may be seen." (Encyclopaedia of Medical Imaging Volume III:1). Melorheostosis usually presents in a sclerotomal distribution. [edit] Clinical Presentation Early clinical findings include joint swelling, limitation of motion and pain. Contractures, ligamentous and tendinous shortening and even soft tissue involvement may ensue.(Monomelic melorheostosis of the hand by Brenner, Wannske). in Diagnostic Radiology/Musculoskeletal Imaging/Dysplasia Basic/Melorheostosis From Wikibooks, the open-content textbooks collection