The Scleroderma is a chronic inflammatory disease of the connective tissue with involvement of the skin and other organs. It can be a manifestation of various disorders and occasionally acroosteolysis in the phalanges. Acroosteolysis is characterized by bone resorption or destruction in the phalanges, while the base is preserved. The pathogenesis of acroosteolysis in patients with scleroderma is a blood-flow disorder that is mainly associated with an abnormal accumulation of collagen in all tissues, microangiopathy and infections in the phalanges. The phalanges in patients with scleroderma are prone to continuous infections as a felon or skin ulcers due to atrophy of the subcutaneous tissue, dry and sclerotic skin, or a disorder of the immune system. We experienced a patient who had acroosteolysis with scleroderma of the phalanges, and this was associated with a felon. We report on this case along with a brief review of the literature.
Acro-Osteolysis ; Atrophy ; Bone Resorption ; Collagen ; Connective Tissue ; Humans ; Immune System ; Skin ; Skin Ulcer ; Subcutaneous Tissue

Acro-osteolysis is characterized by bone resorption in the fingers and toes and can occur in several diseases. Occupational exposure to polyvinyl chloride polymers, thermal injury, and repetitive mechanical injury can also cause acro-osteolysis. The pathophysiology of the disease is unknown, but a unifying theme of vascular and mechanical injury is presented. Signs include Raynaud's phenomenon, bone loss in the hand and cold sensitivity. We report the case of a 67-year-old woman who presented with acro-osteolysis after being burned repeatedly.
Acro-Osteolysis ; Aged ; Bone Resorption ; Burns ; Cold Temperature ; Female ; Fingers ; Hand ; Humans ; Occupational Exposure ; Peripheral Nervous System Diseases ; Polymers ; Polyvinyl Chloride ; Toes

Acro-osteolysis is characterized by bone resorption in the fingers and toes and can occur in several diseases. Occupational exposure to polyvinyl chloride polymers, thermal injury, and repetitive mechanical injury can also cause acro-osteolysis. The pathophysiology of the disease is unknown, but a unifying theme of vascular and mechanical injury is presented. Signs include Raynaud's phenomenon, bone loss in the hand and cold sensitivity. We report the case of a 67-year-old woman who presented with acro-osteolysis after being burned repeatedly.
Acro-Osteolysis ; Aged ; Bone Resorption ; Burns ; Cold Temperature ; Female ; Fingers ; Hand ; Humans ; Occupational Exposure ; Peripheral Nervous System Diseases ; Polymers ; Polyvinyl Chloride ; Toes

Hajdu-Cheney syndrome (HCS) is a rare skeletal dysplasia that is characterized by acroosteolysis of the distal phalanges, distinctive craniofacial and skull changes, dental abnormalities and generalized osteoporosis. The clinical and radiologic characteristics are variable and these characteristics progress with age. This syndrome shows autosomal dominant inheritance with sporadic cases. The genetic defects or molecular pathogenesis of HCS are still unknown. We experienced a case of Hajdu-Cheney syndrome in a 20-year-old man who had generalized osteoporosis with multiple non-traumatic spine compression fractures. He had acroosteolysis of the hands and feet, wormian bones in the skull, facial dysmorphism (mid-facial flattening, micrognathia and bushy eyebrows), a high arched palate, malocclusion and short dental alveolar processes. HCS was diagnosed based on the clinical and radiologic evidence. For the differential diagnosis, we excluded the other possible causes of the acroosteolysis and wormian bones, including hyperparathyroidism, osteogenesis imperfecta, hypophosphatemia and mandibuloacral dysplasia. The specific treatment of HCS is unknown, but case reports with bisphosphonate treatment have been reported.
Acro-Osteolysis ; Alveolar Process ; Diagnosis, Differential ; Foot ; Fractures, Compression ; Hajdu-Cheney Syndrome ; Hand ; Humans ; Hyperparathyroidism ; Hypophosphatemia ; Malocclusion ; Osteogenesis Imperfecta ; Osteoporosis ; Palate ; Skull ; Spine ; Wills ; Young Adult

Osteolytic disease of the bones have a myriad range of aetiology. One rare cause is Gorham’s disease or disappearing bone disease. This disease is a diagnosis by exclusion using correlation made with clinical presentation, radiological findings and histopathological confirmation. Although many different therapies have been advocated, none have been successful in fully controlling this disease. We present a case that was detected in a Chinese lady using diagnostic imaging, confirmed with computed tomography guided biopsy and successfully treated with joint reconstruction using endoprosthesis.
Osteolysis, Essential

Multicentric disappearing bone disease, or Gorham disease, is a rare entity. A middle age woman, presented to us with left sided antalgic gait and severe bony deformity of her left knee. Radiograph revealed massive bone defect of the medial condyle of the left tibia with subluxation of the knee joint. She was scheduled for knee replacement in six months. However, she developed another lesion over the right hip that typically mimicked the disease progression of disappearing bone disease. The right femoral head vanished progressively within three months without significant history of infection or trauma. Subsequent bone biopsy of the right femoral head and left tibia condyle confirmed the diagnosis. Total knee replacement was carried out for her left knee. She remained pain free on her left knee. A year later, after confirming by sequential radiographs that the osteolysis had stopped, total right hip replacement was performed. Five years later, she remained pain free and both the arthroplasties were stable.
Osteolysis, Essential

Although the purpose of orthodontic treatment is to increase the function and esthetics of the jaws along with increasing stability, there are many side effects during the treatment itself, such as root resorption and alveolar bone resorption. Such resorption of the apical root is unpredictable, and may even proceed into the dentin layer. Once the process has begun, it is irreversible. By evaluating the effect of many oral habits, especially that of nail biting, in correlation with the root and the periodontal tissues, the appropriate biomechanics for orthodontic treatment can be taken into consideration, along with the possibility of root resorption and alveolar bone loss during orthodontic treatment, and any legal problems that might occur. Among the male and female patients of the ages 10~15 without skeletal deformity, 63 were chosen as the experiment group with known nail biting habits at time of examination, and within the same age group without nail biting habits as the control. After the orthodontic treatment, number of the experiment group was 31 and the control group was 22. The periapical radiographies of anterior teeth were taken and the assesment of the root length and alveolar bone level were taken before(T1) and after(T2) the orthodontic treatment. The results from this study were as follows : 1. Before the orthodontic treatment, average crown-to-root ratio of the experimental group showed noticeably high values in 4 maxillary incisors and mandibular right central incisor. 2. Before the orthodontic treatment, comparing the root length, maxillary and mandibular right central incisors and both mandibular incisors had a smaller value in the experimental group. 3. Before the orthodontic treatment, comparing and evaluating the alveolar bone loss measured from the cemento-enamel junction to the alveolar bone crest, some crestal bone of the experiment group showed greater loss than the control. 4. After the orthodontic treatment, there was shortening of the root length and loss of the crestal bone in both groups. 5. After the orthodontic treatment, the changes of C/R ratio and the shortening of root length were significantly high in the experimental group. 6. After the orthodontic treatment, the level of alveolar crestal bone showed greater loss in the experimental group.
Alveolar Bone Loss ; Bone Resorption ; Congenital Abnormalities ; Dentin ; Equidae ; Esthetics ; Female ; Humans ; Incisor ; Jaw ; Male ; Nail Biting* ; Root Resorption ; Tooth

PURPOSE: The marginal bone levels around implants following restoration are used as a reference for evaluating implant success and survival. Two design concepts that can reduce crestal bone resorption are the microthread and platform-switching concepts. The aims of this study were to analyze the placement of microthreaded and platform-switched implants and their short-term survival rate, as well as the level of bone around the implants. METHODS: The subjects of this study were 27 patients (79 implants) undergoing treatment with microthreaded and platform-switched implants between October 2008 and July 2009 in the Dental Hospital of Yonsei University Department of Periodontology. The patients received follow-up care more than 6 months after the final setting of the prosthesis, at which time periapical radiographs were taken. The marginal bone level was measured from the reference point to the lowest observed point of contact between the marginal bone and the fixture. Comparisons were made between radiographs taken at the time of fixture installation and those taken at the follow-up visit. RESULTS: During the study period (average of 11.8 months after fixture installation and 7.4 months after the prosthesis delivery), the short-term survival rate of microthreaded and platform-switched implants was 100% and the marginal bone loss around implants was 0.16+/-0.08 mm, the latter of which is lower than the previously reported values. CONCLUSIONS: This short-term clinical study has demonstrated the successful survival rates of a microthread and platform-switched implant system, and that this system is associated with reduced marginal bone loss.
Alveolar Bone Loss ; Bone Resorption ; Dental Implants ; Follow-Up Studies ; Humans ; Prostheses and Implants ; Survival Rate

Periodontitis is an inflammatory disorder of the periodontium, characterized by destruction of the tooth supporting tissues including alveolar bone and mediated by various pro-inflammatory mediators. Here, we demonstrated that HP08-0106, composed of four crude drugs-Gardenia jasminoides Grandiflora, Angelica gigas Nakai, Rehmannia glutinosa, and Schizonepeta tenuifolia in a weight ratio of 2:2:1:2, perturbs inflammatory responses, osteoclast formation in LPS-induced RAW 264.7 cells and alveolar bone resorption in ligature-induced periodontitis. HP08-0106 decreased the protein level of iNOS and COX2 as well as the secreted level of IL-1beta, indicating that HP08-0106 has antiinflammatory effects. HP08-0106 also inhibited the expression of genes associated with osteoclastogenesis including c-Fos, MMP-9 and TRAP. Moreover, HP08-0106 exhibited a protective effect from alveolar bone loss in ligature-induced periodontitis animal models. Our results strongly suggest that HP08-0106 represent an important therapeutic tool to treat inflammatory disorders associated with bone loss such as periodontitis.
Alveolar Bone Loss ; Angelica ; Animals ; Bone Resorption ; Lamiaceae ; Models, Animal ; Osteoclasts ; Periodontitis ; Periodontium ; Rats ; Rehmannia ; Tooth

Hajdu-Cheney syndrome is a rare, autosomal dominant skeletal dysplasia marked by acro-osteolysis of the distal phalanges and severe osteoporosis. Although there are more than 60 reports published to date, proper treatment and subsequent outcome have been scarce. Herein, we report a progress of anti-resorptive therapy with zoledronic acid, in a woman with Hajdu-Cheney syndrome. Results suggest that anti-resorptive therapy may be important in delaying the progress of osteoporosis and preventing fractures, but not necessarily acro-osteolysis itself.
Acro-Osteolysis/complications/*drug therapy ; Adult ; Bone Density Conservation Agents/*therapeutic use ; Diphosphonates/*therapeutic use ; Female ; Hajdu-Cheney Syndrome/complications/*drug therapy ; Humans ; Imidazoles/*therapeutic use ; Osteoporosis/complications/*drug therapy