Fasciitis-panniculitis in acute brucellosis.
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Biomedical subjects
Publications and source records attributed to J E Naschitz.
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The multitude of classic manifestations of paraneoplastic thromboembolism (TE), including new aspects, are reviewed. This emphasizes the complexity of the situation that the physician faces in dealing with paraneoplastic TE. Unexplained TE may serve as a hint for the presence of a hidden tumor. However, efforts to uncover such an underlying malignancy often are unrewarding. The view has been expressed that it is inappropriate to conduct an extensive search for an occult neoplasm unless there are more specific indications. A recent study defined clues that might separate patients with TE and occult cancer from those unaffected by a malignant neoplasm. The study of the hemostatic alterations in patients with cancer underscores the triple role of cancer cells in the pathogenesis of TE: injury to the endothelial lining of blood vessels, activation of platelets, and activation of blood coagulation and depression of anticoagulant functions. The failure of standard anticoagulant treatment in many instances is better understood. Novel approaches to treatment include low-molecular-weight heparin(s) for long-term administration or, alternatively, the initial placement of a Greenfield filter in the vena cava instead of anticoagulant therapy. Either of these may provide superior results in comparison to standard heparin treatment.
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.
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The hypothesis was tested that cancer, by virtue of it being a thrombotic diathesis, may enhance ischemic heart disease. Because cancer therapy may precipitate thromboembolism, the authors focused on patients with occult neoplasia before therapy. Occult cancer was defined as a time period of 2 years before a cancer diagnosis was established. Data obtained from the files of patients with the diagnosis of malignant tumors and admitted to a general hospital during a 3-year period were reviewed for coronary risk factors, coronary events, and characteristics of cancer. Indices of coronary instability were studied: the incidence of first coronary events, the incidence of all coronary events, and the coronary events burden. These indices were calculated for 366 patients with cancer (from the Department of Medicine files of 166 consecutive patients with cancer of several primary sites and from the surgical ward files of 100 consecutive patients with colorectal cancer and 100 consecutive patients with cancer of the prostate or bladder) and for 100 patients with benign prostatic hypertrophy. The patients with benign prostatic hypertrophy served as controls. A steep and statistically significant increase in coronary instability indices was observed in all groups of patients with cancer in the 2-year period before cancer diagnosis in comparison with the coronary instability indices of control patients (P less than or equal to 0.05 to 0.0001). Patients with colorectal cancer presented the highest indices in the 2-year period before cancer diagnosis, with unstable ischemic heart disease being reported in 18% and first coronary events in 10%. The coronary events burden was 0.92. The lowest indices among patients with cancer were recorded in those with prostatic and bladder cancer. Unstable ischemic heart disease occurred in 6% and first coronary events in 4%. The coronary events burden was 0.36. The indices were several times lower for control patients than for patients with cancer. Unstable ischemic heart disease occurred in 3% of control patients, and first coronary events in 2%. The coronary events burden was 0.15. Other possible etiologic factors, particularly the known coronary risk factors and anemia, were not statistically related with an increased risk of coronary events in the 2-year period before cancer diagnosis. Based on these epidemiologic data, it appears that there may be an association between occult cancer and coronary events.
The files of 122 patients treated for at least 6 months with bezafibrate, a second generation fibric acid derivative, were reviewed. Our indications for bezafibrate treatment included cases of type IIb, IV and V hyperlipidemia which did not respond to a serious dietetic trial. Mean decrease in plasma triglycerides was 45% and in cholesterol 1012%, while in HDL-cholesterol there was a mean increase of 8%. The drug was usually well tolerated. Thorough review of patient files for side effects revealed gastrointestinal disturbances in 6.5%. There was a decrease in libido in 4%. 2 patients developed gynecomastia, 1 abnormal liver function tests and 1 severe myositis. All side effects were fully reversed on discontinuing the drug. Bezafibrate seems to be well-tolerated and suitable for treating type IIb, IV and V hyperlipidemia unresponsive to diet.
Localized nodular myositis was recognized in an elderly man six months prior to the diagnosis of Hodgkin's disease. Meticulous search of the muscle specimen failed to disclose tumorous involvement. The possible paraneoplastic nature of localized nodular myositis in this patient is discussed.
Thromboembolism frequently complicates advanced cancer. The incidence of TE as one of the initial manifestations of occult cancer and the diagnostic value of TE as a signal of a possible unrecognized tumor were the subjects of recent studies. TE may precede the diagnosis of cancer by several months or years. The polymorphism of manifestations of paraneoplastic TE has been described previously. An accelerated course of intermittent claudication and of ischemic heart disease has been described in patients with cancer and probably represents additional variants of Trousseau's syndrome. Recently, clues for the presence of occult neoplasms in patients with TE have been proposed. Their value in the stratification of patients needs to be established in prospective studies. That cancer may be responsible for a precipitated course of coronary or peripheral arterial disease raises the question of whether work-up is recommended to uncover a silent malignancy in a patient who has been referred for treatment of these severe ischemic syndromes.
The term fasciitis-panniculitis syndrome (FPS) is proposed as a novel compilation encompassing several disorders, common to which is subcutaneous induration caused by cicatrizing fasciitis as well as septal and lobular panniculitis and perimysial fibrosis. Included herein are Shulman's eosinophilic fasciitis, morphea profunda, lupus profundus, venous lipodermatosclerosis, toxic oil syndrome, altered tryptophane-related eosinophilic myositis, graft-versus-host reaction, and fasciitis reactive to subjacent basal cell carcinoma. FPS should be differentiated from scleroderma, which primarily affects the dermal structures and in which arterioles are injured. In contrast, vasculopathy of the subcutaneous medium-sized veins accompanies the hypodermal lesions of FPS. The importance of recognizing and grouping these disorders lies in their different histopathology, characterization as reactive phenomena, enhanced responsiveness to treatment, and better prognosis than scleroderma. In view of the excellent prognosis of FPS, steroid treatment is not warranted. Long-term therapy with cimetidine appears to benefit the majority of patients.
The authors report on a patient with an undescribed constellation of lymph nodal plasmacytosis, perinodal epithelioid cell granulomas and fibrosis as well as anti-Sm and anti-phospholipid antibodies. The illness does not meet the criteria of well-known nosologic entities, but it is thought to represent an unrecognized nosological entity within the group of undifferentiated immune disorders.
An elderly woman receiving long term treatment with prednisone and azathioprine for cryptogenic chronic active hepatitis developed granulomatous lipophagic panniculitis and temporal arteritis. The lymphoplasmahistiocytic inflammatory reaction pattern is common to this patient's three diseases. It is suggested that an aberration of the defence mechanisms, immunological or otherwise, is responsible for this unusual occurrence. The triple association of chronic active hepatitis, granulomatous panniculitis and temporal arteritis has not been reported previously.
The fasciitis-panniculitis syndrome is histologically characterized by fibrous thickening and inflammation of the subcutaneous septa, fascia and perimysium. These morphological features were the common denominator in 13 patients with Shulman's eosinophilic fasciitis, scleroderma profunda, venous lipodermatosclerosis, induration following recurrent erysipelas and erysipeloid erythema nodosum. Small to medium-sized vessel phlebitis was found in the subcutaneous and perimysial tissues of 11 of the patients and subcutaneous phlebosclerosis was encountered in all cases. These data contrast with the low incidence of phlebopathy reported by others in these disorders.
A solitary cervical metastasis of a typical carcinoid tumor was found in the subcutaneous tissue of an asymptomatic 38-yr-old woman. Investigations failed to disclose the primary site until the 5th yr, when she presented with carcinoid syndrome. Multifocal ileal carcinoid tumors were resected and debulking of abdominal metastases performed. Interferon and somatostatin analogue treatment resulted in remission. Solitary cervical metastasis is an exceedingly rare initial manifestation of a mid-gut carcinoid tumor, and poses a therapeutic dilemma. There are no directions in the literature as to whether a "wait-and-see" approach or exploration surgery is the preferred management when one is confronted by a cervical metastasis of typical carcinoid tumor of unknown primary site.
Massive and diffuse subcapsular fibrosis of the liver was detected in two patients with chronic liver disease. The subcapsular layer consisted of inflamed fibrous tissue containing rare portal tracts and myriads of bile ducts, indicating loss of subcapsular hepatocytes and their replacement by connective tissue. In the early evolutive phase, massive and diffuse subcapsular fibrosis of the liver appears on computed tomography (CT) scan as a low-density band, which merges with the hepatic parenchyma and is enhanced by contrast on dynamic imaging; it is undetectable by ultrasonography. In the advanced phase, it is easily recognizable by ultrasonography as a perihepatic sonolucent band and by CT scan as a low-density band, which is well demarcated from the hepatic parenchyma and is not enhanced with the dynamic technique.
Two patients who presented with discomfort in the calves while walking down a slope were diagnosed as suffering from eosinophilic fasciitis of the lower limbs. Following therapy of the underlying disease, this peculiar discomfort remitted concomitantly with the disappearance of the subcutaneous abnormalities. The authors posit that downslope claudication of the calves in eosinophilic fasciitis constitutes an additional variant of claudication of the lower limbs.