MR imaging of pachydermoperiostosis. (1/31)

A case of pachydermoperiostosis who demonstrated the whole syndrome (pachyderma, periostitis, and cutis verticis gyrata) is presented, and the Magnetic Resonance Imaging (MRI) appearances of the long bone and scalp changes are demonstrated. MRI of the cruris demonstrated fluffy periosteal new bone formation that encroached on the medullary cavity as well as expansion of the diaphysis. Cranial changes included thickening of the diploe associated with diminished signal of the intradiploic fat, and thickening of the scalp with furrowing.  (+info)

Hypertrophic osteoarthropathy can indicate recurrence of Whipple's disease. (2/31)

We report the case of a patient with Whipple's disease (WD) who developed hypertrophic osteoarthropathy (HOA) characterized by digital clubbing, periostosis of the tubular bones, and polysynovitis. The HOA disclosed the recurrence of the patient's WD, since polymerase chain reaction (PCR) analysis clearly demonstrated the presence of Tropheryma whippelii in the synovial fluid from the patient's left knee. Initiation of appropriate antibiotic therapy resulted in complete healing of all clinical rheumatologic manifestations within 2 months and in disappearance of radiographic bone changes at 7-month followup. We suggest that HOA be included within the spectrum of rheumatologic manifestations of WD, and that an evaluation for WD should be considered in patients, especially middle-aged men, presenting with HOA even without gastrointestinal symptoms. PCR analysis may be useful in accurate diagnosis and management of early WD with unusual clinical manifestations, and may contribute to decreased morbidity and mortality.  (+info)

A case of hypertrophic osteoarthropathy associated with nasopharyngeal carcinoma in a child. (3/31)

Hypertrophic osteoarthropathy is characterized by clubbing of the digital tips and periosteal reaction of long bones. Most of the cases are associated with malignancy or other conditions such as congenital heart disease, liver cirrhosis, pulmonary fibrosis, biliary atresia, and gastrointestinal polyps. Hypertrophic osteoarthropathy associated with malignancy is rare in children. A few cases of hypertrophic osteoarthropathy in children with nasopharyngeal carcinoma have been reported, however, there has been no report of such case in Korea. We present a case of hypertrophic osteoarthropathy associated with nasopharyngeal carcinoma with lung metastasis in a 14-yr-old boy. In this case, hypertrophic osteoarthropathy regressed after intensive chemotherapy, but subsequently the patient died of progressive lung metastasis.  (+info)

Fibromatosis of the hand associated with EMO syndrome: a case report. (4/31)

BACKGROUND: EMO syndrome, defined as a triad including exophthalmus, pretibial myxedema and osteoarthropathia, is a rare condition in patients suffering from hyperthyreosis. CASE PRESENTATION: We here describe an interesting case of EMO syndrome associated with unilateral fibromatosis of the hand and an initial stage of generalized myxedema of the skin. To our knowledge a similar case has not yet been described in literature though reports about associated fibromatosis, e.g. located retroperitoneally, already exist. Familiar explanations include its initiation by autoimmune processes or aberrant T-cell cytokine stimulation leading to an overwhelming production of glycosaminoglycans. CONCLUSION: Interpreting our case in context with previous reports we conclude that associated fibromatosis induced by autoimmune processes may affect a variety of different localizations and therefore requires careful monitoring. A therapeutical attempt by using UVA1 irridation for pretibial myxedema remained without a satisfying regression.  (+info)

Bone abnormalities and severe arthritis in pachydermoperiostosis. (5/31)

Two patients with pachydermoperiostosis were studied in whom the predominant features at presentation were severe and disabling knee and ankle joint pain in association with distal long bone pain. Analysis of synovial fluid from the knee joints showed non-inflammatory changes. In one patient a bicortical iliac crest bone biopsy specimen, taken after labelling with demeclocycline, showed appositional rates which were increased in cortical bone but reduced in trabecular bone. This mismatching resulted in trabecular osteoporosis, which occurred in association with increased numbers of osteoclasts, findings which suggest differential functional changes affecting the two bony envelopes.  (+info)

Absence of an association between HLA typing in cystic fibrosis arthritis and hypertrophic osteoarthropathy. (6/31)

Fourteen patients with cystic fibrosis arthritis and eight patients with cystic fibrosis and hypertrophic osteoarthropathy were typed for HLA-A, B, C, DR, and DQ antigens and were compared with age and sex matched controls with cystic fibrosis. The diagnosis of cystic fibrosis arthritis and hypertrophic osteoarthropathy was confirmed by radiography and bone scanning. The prevalence of HLA-A, B, C, D, antigens in the cystic fibrosis group (44 patients) did not differ from that in the control group. A comparison between patients with cystic fibrosis arthritis or hypertrophic osteoarthropathy and their respective controls did not show any significant differences in HLA prevalence. It is concluded that HLA antigens may not be a factor in the susceptibility of patients with cystic fibrosis to cystic fibrosis arthritis or hypertrophic osteoarthropathy.  (+info)

Unusual associations of pachydermoperiostosis: a case report. (7/31)

Primary hypertrophic osteoarthropathy (HOA), or pachydermoperiostosis, is a rare benign disorder of unknown etiology. It is characterized by clubbing, periosteal reaction, and thickening of the skin. Disease usually progresses slowly, and natural arrest may occur. Reported herein is the case of a 28-year-old male patient with progressively increasing swelling of large joints of lower limbs with severe anemia. He was diagnosed as a case of pachydermoperiostosis with myelofibrosis, which is a rare association. The development of myelofibrosis makes primary HOA a disease with unfavorable outcome.  (+info)

Ptosis caused by pachydermoperiostosis. (8/31)

Pachydermoperiostosis is a rare inherited disorder which presents with finger clubbing, facial enlargement, and periostitis. A case is described in which surgery for ptosis was performed and the differential diagnosis of the condition is discussed. The histological and ultrastructural appearances of the eyelids show sebaceous gland hyperplasia and excessive deposition of mucin in the dermis and would suggest that pachydermoperiostosis may be an example of a cutaneous mucinosis.  (+info)