Elimelech Zuckerman

Rheumatic syndromes: Clues to occult neoplasia

Rheumatic disorders associated with cancer include a variety of conditions, most of which have no features distinguishing them from idiopathic rheumatic disorders. It is generally held that an extensive search for occult malignancy in most rheumatic syndromes is not recommended unless the case is accompanied by specific findings suggestive of malignancy. Within the past year information has accumulated on the role of long-standing rheumatic disorders as premalignant conditions and the role of autoantibodies as screening tests for occult cancer. The present article discusses cancer-associated rheumatic syndromes, calls attention to aspects that may suggest the presence of a hidden cancer, and examines the role of laboratory tests as clues of a possible neoplastic etiology of those syndromes.

The Fasciitis-Panniculitis Syndrome: Clinical Spectrum and Response to Cimetidine

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 Shulmans 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.

Systemic sclerosis sine scleroderma: is it always the same disease? Report of three patients and discussion

Abstract The recent description of a large cohort of patients with the diagnosis of systemic sclerosis sine scleroderma (ssSSc) provided significant progress in our understanding of this entity. The prognosis of patients with ssSSc is, however, very variable, from benign in most cases to rapidly disabling in others. By reporting three new cases and analyzing previously published data, we discuss possible subsets and variants of the disease form.

FASCIITIS‐PANNICULITIS IN ACUTE BRUCELLOSIS

A 25-year-old woman villager, who previously enjoyed good health, was hospitalized 2 weeks after she first noticed malaise, fever, and bilateral calf swelling. Two days prior to admission, her 3-year-old son was hospitalized with a febrile illness in the Department of Pediatrics, A tube agglutination titer of 1/320 and, later, blood cultures positive for Brucella melitensis established tbe diagnosis of brucellosis, Tbe patient conceded to her familys habit of drinking unpasteurized goat milk. Except for bepatosplenomegaly and bilateral calf swelling, physical examination was unrewarding, Leukopenia with relative lympbocytosis was found. Initially, Brucella antibodies were undetectable, Tbe tube agglutination test, repeated 10 days later, was positive for Brucella melitensis at a titer of 1/80, Doxicillin and rifampin treatment was begun, Tbe distal thirds of the calves were swollen. While tbe color and temperature of the skin were normal, the subcutaneous layers at tbese sites were indurated; the skin fold measured 5 cm (Fig, 1), The proximal border of tbe swelling was well demarcated, while the distal aspect merged imperceptibly witb the normal integument of the foot. The bistologic changes of a full thickness biopsy were confined to the subcutis and fascia, tbe skin and muscle being normal, Tbe subcutaneous septa and fascia were fibrotically thickened and contained sparsely scattered lymphoyctes. The fat tissue lobules were heavily infiltrated by lymphocytes admixed witb few bistiocytes and plasma cells (Fig, 2), There were some small and irregularly shaped foci of necrosis in the fat tissue. Following initiation of antibiotic medication, defeverescence occurred witbin a week and the subcutaneous induration progressively diminished. No residual changes were palpated after 6 weeks, at the termination of treatment. Figure 1. Photograph of the calves taken during the second week of illness. Both calves are swollen. The skin fold measured 5 cm.

Spontaneous Perinephric Hemorrhage in a Middle-Aged Diabetic Woman

Spontaneous perinephric hematoma is a rare condition that is usually caused by benign and malignant renal tumors, vascular abnormalities and inflammatory disorders. However, a few patients in whom there is no apparent underlying disease are described as having idiopathic spontaneous perinephric hematoma. We report on a middle-aged patient with diabetes mellitus who was hospitalized for sudden onset of right upper flank pain. A large tender mass was palpable in the right upper abdominal quadrant. The hematocrit decreased from 32% on admission to 23% during the following hours. Computerized tomography of the abdomen revealed a large perinephric mass, which was denser than the adjacent renal parenchyma and hypodense after intravenous iodine injection, findings which were compatible with perinephric hematoma. The patient underwent exploratory laparotomy because of hemodynamic instability. Uncontrollable bleeding from a discolored apical renal lesion led to nephrectomy. On pathological examination this apical lesion proved to be a renal infarct with considerable bloody inhibition of perirenal tissues. Extensive search for an underlying disease that led to the infarction was unsuccessful.

Rheumatoid-like Arthritis Associated with Hepatitis C.

Rheumatoid arthritis is a clinical entity whose etiology is unknown, although an infectious cause has been suggested. We describe three patients diagnosed to have a rheumatoid arthritis-like disease that was chronic but nonerosive and unresponsive to nonsteroidal anti-inflammatory drugs. The patients were females aged 30, 69 and 70 years who had morning stiffness of more than 30 minutes and symmetric polyarthritis for periods of 4, 6, and 20 months, respectively. A laboratory screening revealed minunal liver enzyme elevations, whereas acute phase reactants were consistent with systemic inflammation, and the presence of rheumatoid factor and cryoglobulins was detected. Serology was positive for hepatitis C virus antigens. Liver biopsies revealed hepatitis and early cirrhosis. Alpha-interferon therapy was associated with clinical improvement of arthritis in two cases. It is suggested that hepatitis C infection may be associated with rheumatoid-like arthritis.

Case Report: Extensive Pulmonary and Aortic Thrombosis and Ectasia

Progressive shortness of breath developed in an elderly woman with a 25-year history of recurrent superficial phlebitis and hemoptysis. Extensive mural thrombosis and ectasia of the large and medium-sized pulmonary arteries and aorta were revealed on echocardiography and computerized tomography. The patient died 2 months later. On autopsy, the gross morphologic findings were similar with those observed by imaging. Histologically, there was mild inflammation in the intima and media of the aorta and the large pulmonary arteries, consistent with nonspecific arteritis. The extensive thrombosis and ectasia of the pulmonary arteries and aorta differ from previously published cases and cannot be assigned to a known nosologic entity. Two alternative explanations are proposed. First, an endothelial disorder was responsible for a diffuse vasculopathy that involved veins, pulmonary arteries, and aorta. Second, a vasculopathy of the Hugh-Stovin type, characterized by phlebitis and pulmonary thromboembolism, caused pulmonary hypertension and low cardiac output. The low flow state favorized aortic thrombosis and, at the site of interaction between the clot and the arterial wall, arteritis developed as an epiphenomenon, which induced arterial dilatation. Combined idiopathic pulmonary artery and aortic thrombosis and ectasia is rare and calls for corroboration of sporadic observations such as the current one.

Case report: lymphadenopathy, perinodal granulomatosis, and immunologic aberrations.

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.

Diffuse Lymphangioma with Intrathoracic Involvement

A case of a young girl with oligosymptomatic multiple cystic lesions of the chest is presented. The chest lesions were found on routine chest X-ray when she was admitted because of meningococcal meningitis. As the lesions increased in size, surgery was performed; the operative specimens were identified as lymphangioma. This is a rare disorder, which presents difficulties in diagnosis and treatment. Its benign nature is challenged by its tendency to recur and the need for repeated operations.