Striated hypertrophy of limbs

Introduction Melorheostosis is also known as bone candle disease, single-arm candle-like hyperosteogeny, and Leri bone sclerosis candle bone. For a rare shape of the localized bone hyperplasia, from the X-ray film, it seems that the molten wax flows down the backbone. It is a congenital malformation that is rare. No hereditary and family history. The onset begins in children and is equal for men and women. Most of them infringe on one limb, and the lower limbs are more common in the upper limbs.

Cause

Etiology

The etiology of this disease is unknown, and it is believed that multiple congenital malformations are a kind of periosteal bone dysplasia caused by subperiosteal telangiectasia. There are reports of familial brittle bone sclerosis in the literature, so it is thought that it may be caused. Caused by a secondary mutation in the isolated gene of the familial fragility syndrome. No hereditary and family history.

[pathological changes]

The epithelium and endosteal hyperplasia are irregularly hardened. Microscopically, the bone hyperplasia area is a tight lamellar bone, arranged disorderly, Harvard's tube is distorted, immature and mature bone tissue interweave, osteoblast activity increases, osteoclast activity decreases, so new bone formation. Other structures are normal.

an examination

X-ray examination has irregular bone hyperplasia on one side of the backbone. The shape of the backbone is destroyed, as if the wax of the burning candle is flowing down from the side. The hyperplastic bone has no structure, and the epiphysis and short bone often appear as spots, which can surpass the joint to invade the distal bone, but does not invade the joint surface. The pelvis and scapula also showed increased density and spots, skulls, spines and ribs are rare.

Differential diagnosis

For typical cases, because of their special performance, diagnosis is not difficult, and some cases need to be differentiated from the following diseases.

1. Osteosclerosis (marble osteopathy): The cause of osteopetrosis is not clear, and may be related to abnormal bone resorption, resulting in excessive deposition of calcium salts in the bone. The appearance is marble or ivory, and the fragility is increased. The disease has a family history and is more common among children who are married to close relatives. Some people think it is a hereditary disease. The disease is divided into two types, light type is dominant inheritance, and heavy type is recessive inheritance.

2, bone spot disease (vulnerability osteosclerosis): Osteopoikilosis (Osteopoikilosis) is a rare disease, according to the statistical incidence of insufficient 1 / 10 million people, without any clinical symptoms. It is named because it has diffuse speckled dense bone inside the bone.

3, bone fiber dysplasia: the cause of bone fiber dysplasia is unknown, some people think that it is caused by internal spasm disorder, others are thought to be formed by abnormal bone development, but mainly may be malnutrition or serious loss of individual nutrients The bone structure caused by dysplasia. This disease occurs mostly in adolescents. 11-30 years old is a high-risk age range. The most common site of the limb is the tibia, femur, and jaw bones, followed by the ribs and skull.

4, inflammatory osteosclerosis, etc.: it does not produce abscesses, dead bones and the formation of fistulas. There are a few wounds that may have some pus and granulation tissue, and the culture may have Staphylococcus aureus growth. The systemic symptoms are not obvious, the local is sometimes asymptomatic, or there is an inflammatory reaction such as swelling and pain. The tenderness and pain are more severe in the night than in the daytime. The course of the disease develops prolonged, and the trauma or other diseases may be exacerbated, but the majority of the joints are not affected.

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