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1/66. Correction of eyelid anomalies in pachydermoperiostosis.

    PURPOSE: Pachydermoperiostosis, or primary hypertrophic osteoarthropathy, is a rare disease, characterized by pachydermia, acropathy, and periostosis. The authors propose the surgical correction of the eyelid anomalies in one stage. methods: A 30-year-old patient with moderate blepharoptosis (3 mm) and increased vertical and horizontal dimensions of the upper eyelids underwent fusiform transverse excision of skin, orbicularis muscle, and tarsus; shortening of the levator palpebrae superioris apeoneurosis by 13 mm; and 8-mm-wide wedge excision. RESULTS: Histologic findings included hyperplasia of the tarso-conjunctival plate, obstructive cystic dilatation of the sebaceous glands, extensive fibrosis, and granulomatous reaction. Satisfactory functional and aesthetic results were observed after surgery. CONCLUSION: Upper eyelid anomalies in pachydermoperiostosis can be corrected in a single stage bilaterally with good results. ( info)

2/66. MR imaging of pachydermoperiostosis.

    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)

3/66. A rare case of acromegaly associated with pachydermoperiostosis.

    Pachydermoperiostosis (PDP) is a rare syndrome manifested clinically by finger clubbing, extremity enlargement, hypertrophic skin changes, and periosteal bone formation. The pathogenesis of the disorder has not been clarified and few endocrine abnormalities were apparent. We report here a 58-year-old man with acromegaly associated with PDP, the features of clubbed fingers, coarse skin, and cutis verticis gyrata. acromegaly due to GH-producing pituitary adenoma was confirmed in endocrinological and pathological studies. ( info)

4/66. Hypertrophic osteoarthropathy can indicate recurrence of Whipple's disease.

    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)

5/66. Primary pachydermoperiostosis: a case report.

    Pachydermoperiostosis (PDP), a rare genodermatosis, occurred in a 38-year-old Indian male. He presented with progressive thickening of the skin on the face and scalp of 15 years duration. Widening of his wrists and ankles and broadening of the fingers and toes had also developed since then. He was born of a consanguineous marriage and had no family history of a similar disorder. He had the typical findings of complete form of PDP including cutis verticis gyrata, coarse facial features, clubbing of the digits in the skin, and periostosis and cortical thickening at the distal ends of long bones of the extremities and small bones of the hands and feet. PDP has two different forms--primary and secondary. These two entities are differentiated by family history and presence or absence of a primary lesion, usually in the lungs. Clinically, in secondary PDP, the cutaneous findings (pachydermia, seborrhoea, oiliness) are less severe than primary PDP; osteoarthropathy is more severe and painful in secondary PDP, especially with congenital heart disease. The present case was suffering from primary PDP that had expressed itself in its complete form. ( info)

6/66. Thymic carcinoma, systemic lupus erythematosus, and hypertrophic pulmonary osteoarthropathy in an 11-year-old boy: a novel association.

    Thymic carcinoma is exceptionally rare in children and it has never previously been associated with autoimmune disorders. The authors report the case of an 11-year-old boy with thymic carcinoma, hypertrophic pulmonary osteoarthropathy, and an autoimmune disease that resembled systemic lupus erythematosus. To their knowledge, this is the first case of such complex clinical findings. The tumor was of high grade histologically and the boy died after 1 year, in spite of chemotherapy and radiotherapy. A review is presented of the available medical literature on thymic malignancy in childhood. ( info)

7/66. Reactivation of primary hypertrophic osteoarthropathy by bronchogenic carcinoma.

    This paper reports 2 cases of primary hypertrophic osteoarthopathy (PHO) which evolved into secondary hypertrophic osteoarthopathy (SHO) under the influence of bronchogenic carcinoma. The patients had a clinical picture of primary hypertrophic osteoarthropathy but without any signs of disease activity until in the last several months when a malignant bronchopulmonary condition developed. This activated all the symptoms: joint swelling; enlarged fingers and distal forearms and legs; moist palms and soles; unpleasant odour of perspiration; and deeper folds of the forehead and nasolabial furrow. A bone scan showed increased accumulation of the radioisotope in specific regions of the skeleton. To our knowledge, no similar cases have been described in the literature. ( info)

8/66. Periodontal and alveolar bone abnormalities associated with pachydermoperiostosis.

    Pachydermoperiostosis (PDP) is an unusual syndrome manifested clinically by finger clubbing, extremity enlargement, hypertrophic skin changes, and periosteal bone formation. A rare case of pachydermoperiostosis (primary hypertrophic osteoarthropathy) with oral manifestations in a 47-year-old man is presented. The possible correlation between physiological mechanisms of this disease and their influence on oral periodontal tissues and alveolar bone is discussed. ( info)

9/66. Ptosis caused by pachydermoperiostosis.

    A 39-year-old man with pachydermoperiostosis is presented. He had enlarged, thickened upper eyelids that made it difficult for him to open his eyes. Eyelid correction was performed by resecting the excessive skin and orbicularis muscle in a fusiform shape and shortening the levator aponeurosis by plication. A wedge resection of the lateral third of the upper lid shortened the horizontal width and also changed the unpleasant fan shape to a better shape, with a natural curvature of the lid. The authors also performed a 5-mm transconjunctival tarsectomy. Visual impairment was corrected with a satisfactory aesthetic outcome. ( info)

10/66. Familial primary hypertrophic osteoarthropathy in association with congenital cardiac disease.

    It is rare to find congenital cardiac disease in association with familial primary hypertrophic osteoarthropathy. We have now encountered three siblings, two of whom had digital clubbing, patent arterial ducts and delayed closure of the cranial fontanels. The third infant was unusual in that there was no clubbing, or cranial abnormality, despite a small ventricular septal defect. To the best of our knowledge, this association has not previously been observed. ( info)
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