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Progressive diaphyseal dysplasia is a rare osteosclerotic dysplasia of the bone which presents clinically with bone pain, muscle weakness and a waddling gait. Radiography, which shows progressive hyperostosis along the long bones forms the basis of the diagnosis. We present a young patient in whom pain and limitation of movement in the shoulder joint area, mimicking capsulitis, were the presenting symptoms of the disease. The diagnostic and the therapeutic approach to this unusual disorder are discussed.

The patellae of 6 male and 2 female, 40 to 70 year-old individuals, who were healthy at the time of their violent death, were assessed by computer-assisted image analysis. The means of the bone density (percentage of bone in the respective field of interest) ranged from approximately 20% to approximately 30% in the central spongiotic zones, from approximately 40% to approximately 80% in the superior and inferior peripheral zones, and approximately 40% to approximately 60% in the subchondral zone. Bone densities were greatest in the lateral parts of the subchondral and spongiotic territories. The bony trabeculae were haphazardly distributed in the central spongiotic zones. They were commonly oriented vertically or parallel to the surface of the patella in the peripheral and subchondral zones. In conclusion, the histomorphometric data presented validate the rationale of reaming the articular aspect of the patella into a dome-shaped configuration with preservation of a circumferential bony bulwark in the preparation for the implantation of a thick polyethylene-based component with a concave undersurface.

Purpose: The aim of this work was to detect nuclear parameters related to the prognosis of patients with stage III, IV or DS neuroblastomas. Methods: Histological sections of 25 operation specimens obtained from children with advanced-stage neuroblastomas were subjected to computer-assisted image analysis. Statistical relationships between nuclear descriptors of the tumor cells and patients' clinical outcome were determined. Results: The coefficient of variability of the mean nuclear area the mean nuclear elongation factor, and the mean nuclear averaged Feret diameter of the neuroblastoma cells were ascertained to be discriminators separating high-grade from low-grade tumors. Conclusions: The histomorphometrically gauged nuclear parameters may help oncologists to assess the prognosis of patients with advanced-stage neuroblastoma.

To explore the potential of histomorphometric analysis in distinction between follicular adenomas and well differentiated follicular carcinomas of the thyroid gland. Their differentiation on routine histological study may be a challenging exercise, being contingent upon the absence or presence of vascular invasion and penetration of neoplastic follicles through the capsule of the tumour.Computer-assisted image analysis was performed to gauge the nuclear area, nuclear Ferret diameter, nuclear regularity factor, nuclear elongation factor, number of nuclear vesicles and total area of all nuclear vesicles in the follicles of 37 adenomas and 36 well differentiated carcinomas. By univariate analysis, these nuclear descriptors (with the exception of the elongation factor) were found to correlate with the benign or malignant nature of the tumours. By multivariate analysis, only the nuclear area, Ferret diameter and regularity factor were ascertained to be significant predictors of malignancy. A fitted logit model correctly predicted 91% of the cancers and 87% of the adenomas.Histomorphometrically gauged nuclear parameters of the tumour cells may reinforce pathologists' decision-making by adding objective and unbiased criteria to their subjective assessment of follicular neoplasms in cases in which vascular or capsular invasion are not detected.

The nuclear parameters of the small lymphocytes in nine cases of small cell lymphomas of the thyroid gland and 17 cases of Hashimoto's thyroiditis were assessed by computer-assisted image analysis. The nuclear area, maximal, minimal and averaged Ferret diameters, perimeter, regularity factor and elongation factor were gauged. Statistically, the nuclear area was ascertained to be the optimum descriptor discriminating between small neoplastic and reactive lymphocytes. Application of a novel variable, combining a nuclear area cut-off value of 14 microm2 with a nuclear averaged Ferret diameter cut-off value of 4.5 micron, allows for the distinction - with a high degree of sensitivity and specificity - between small neoplastic lymphocytes in thyroidal lymphomas and the reactive lymphocytes in Hashimoto's thyroiditis.

A 40y old woman with primary Sjögren's syndrome developed elevated purpura, peripheral neuropathy, muscular tenderness, abdominal pain, heart failure, and convulsive spells. The hallmarks of this disease were high titers of anti-Ro antibodies and low complement levels in the serum, leukocytoclastic small vessel vasculitis in the cutaneous biopsy specimen, and a life threatening clinical course. Echocardiography revealed left ventricular hypokinesis with low ejection fraction, which is unlike the more common features of cardiomyopathy complicating Sjögren's syndrome. The rapidly deteriorating heart failure and other systemic complications remitted on pulse corticosteroid and cyclophosphamide therapy. The pathogenesis of heart failure, which appeared concurrently with vasculitis and was reversed on immunosuppressive therapy, is explained in the context of the systemic disease. Leukocytoclastic vasculitis might be at the origin of this rare variant of acute, severe but reversible cardiomyopathy in pSS.

We describe an experimental model of the fasciitis-panniculitis syndrome, which includes eosinophilic fasciitis and its related disorders. Rats were given a single or repeated subcutaneous injections of saline or a 10% lipase solution. The injection sites were studied histologically and histomorphometrically. With few exceptions, the subcutaneous-fascial unit of the saline-injected rats was normal. An acute necrotizing panniculitis with inflammatory involvement of the adjacent fascia was found 2 days after a single injection of lipase. Three to 6 weeks after a single injection of lipase, the subcutaneous fat tissue and fascia were fibrotically thickened and chronically inflamed. Similar but more advanced alterations had developed in animals killed 3 weeks after three weekly injections of lipase. Progressive fibrotic thickening of the subcutaneous-fascial unit was observed following an increasing number of weekly lipase injections. The acute-phase reaction expresses a pattern of steatonecrotic tissue damage. In the ohronic phase, the fibrotically thickened subcutaneous layers typify a reaction pattern analogous to that of the human fasciitis-panniculitis syndrome. This experimental model permits better appreciation of the disorder's nonspecific pathogenesis and may help in the search for alternative therapeutic modalities.

The clinical presentation of acute appendicitis and the idiopathic granulomatous variety are, as a rule, indistinguishable. Five patients with idiopathic granulomatous appendicitis, treated during past decade, had histological acute and chronic appendicitis abounding with granulomas. Investigations not disclosing a systemic or enteric granulomatous disease and the patients' long-term complete postoperative recovery support the diagnosis of idiopathic granulomatous appendicitis.

An elderly woman underwent jejunectomy for a large, stenosing neoplasm. Histologically, we found an undifferentiated carcinoma with scattered mononuclear and multinuclear macrophages in the intestine and lymph node metastases. The multinuclear cells, being acid phosphatase-positive and CD68-immunoreactive, are referred to as osteoclast-like giant cells. Hepatic secondaries were discovered 6 months postoperatively. The patient succumbed to a chemotherapy-related septic event. We suggest that intratumoral infiltration by mononuclear and multinuclear macrophages expresses one of the body's defense mechanisms against cancer.

The histological features of lipodermatosclerosis and eosinophilic fasciitis and its variants were compared in a prospective study of outpatients attending the vascular clinic and inpatients in the Department of Medicine of a regional university hospital. Main outcome measures examined were swelling and induration of the subcutaneous layers with a stocking distribution in the calves. The inflammatory and fibrosing alterations involving the panniculus adiposus, superficial fascia and perimysium were essentially indistinguishable histologically in patients with lipodermatosclerosis or eosinophilic fasciitis and its variants. The intensity of the subcutaneous induration was related to the underlying nosological entity and the duration of the process. Infectious cellulitis was found to aggravate the clinical symptoms of lipodermatosclerosis. Since the stereotypical inflammatory and fibrosing processes in lipodermatosclerosis and eosinophilic fasciitis and its variants are similar irrespective of the initiating factors, it is suggested that there is a common final pathway in the pathogenesis of both disorders.

A leiomyoma of the fetal membranes was incidentally discovered on examination of a spontaneously expulsed placenta following an uneventful pregnancy and delivery of a healthy neonate. Perusal of the literature uncovered only a single report of a placental leiomyoma.