Systemic lupus erythematosus and inflammatory bowel disease.

We hereby summarize a case reported by the authors, as well as all of the previously reported cases, of patients suffering from both systemic lupus erythematosus (SLE) and inflammatory bowel disease (IBD) that have been published in the English literature. In most cases, SLE was diagnosed prior to IBD, and the latter was of the ulcerative colitis subtype. Interestingly, the first disease diagnosed was almost never active at the time the second disease manifested itself. Patients with both diseases tended to have less photosensitivity, less arthritis, and less serositis than patients with SLE alone. There were no cases with neurological disorders or overt nephritis. All of these patients had anti-dsDNA and there was a tendency towards more anemia and thrombocytopenia. These patients had a relatively benign course with no flare-ups of lupus during follow-up and a favorable course of their bowel disease as well.

Sweet's syndrome and sarcoidosis.

In this review we summarize a number of cases of Sweet's syndrome (SS) associated with sarcoidosis that have been reported in the English literature. In all of the cases, the two disorders were diagnosed simultaneously. Patients with both disorders were younger and had a higher rate of fever than patients with SS alone. In this group of patients, we found a trend toward less skin involvement of the face and trunk, more involvement of the upper limbs, and more atypical skin lesions, particularly papules. The association of the two disorders seems to be more related to a subset of acute sarcoidosis (Lofgren's syndrome). All of the patients in this group had a benign course and self-limiting disease. Thus, SS in association with sarcoidosis could be considered a favorable prognostic factor. Although SS has a high rate of recurrence, no recurrence occurred in this group of patients during follow-up.

Rheumatic fever in the Nazareth area during the last decade.

BACKGROUND: Acute rheumatic fever is considered a relatively uncommon disease in developed countries. Although cases are encountered in the Nazareth area in Israel, no systematic study of this disease has been done in the last 20 years. OBJECTIVE: To study the incidence and characteristics of acute rheumatic fever cases in the Nazareth area during the last decade. METHODS: Cases of acute rheumatic fever diagnosed according to the modified Jones criteria were identified in two hospitals in the Nazareth area during the 10 years. These two hospitals receive about 75% of non-obstetric referrals to the emergency room. Clinical, laboratory and treatment data of these patients were documented and the incidence of disease calculated. The population census in the Nazareth area was obtained from the Central Bureau of Statistics. RESULTS: Forty-four patients, with a mean age of 18 years, were identified. The mean annual incidence was 5 cases/100,000 population. Arthritis was found in 98% of the patients (migratory in 52%) and carditis in 34%, but only one patient had a subcutaneous nodule, and none had either erythema marginatum or chorea. Only one patient with carditis developed heart failure a few months later due to severe mitral stenosis. CONCLUSION: Rheumatic fever in the Nazareth area is still manifest. The mean age of the patients was higher than found previously. In only half of the patients was the arthritis migratory, with other major manifestations of rheumatic fever found only rarely.

Fructose-1,6-diphosphatase deficiency: a rare cause of prolonged prothrombin time.

A 20-year-old woman presented with severe life-threatening metabolic acidosis and hypoglycemia. In addition, her blood tests revealed elevated hepatic enzymes and a prolonged prothrombin time, with a reduction in factor VII activity. After treatment with a glucose and bicarbonate-containing intravenous infusion, there was a dramatic clinical improvement and normalization of the prothrombin time within 2 days. The patient was found to have fructose-1,6-diphosphatase deficiency, a rare metabolic disorder which has not been described previously as causing coagulation defects.

Cytomegalovirus encephalitis in an immunocompetent pregnant woman.

Presented here is the case of an immunocompetent pregnant woman with probable cytomegalovirus encephalitis. The clinical picture was characterized by diffuse headache, drowsiness and the development of an acute confused state. Diagnosis was based on the documentation of recent cytomegalovirus seroconversion with low avidity for the immunoglobulin G class antibodies. Initially, the diagnosis of encephalitis was challenging due to the subtle findings in cerebrospinal fluid and the normal electroencephalograph results. However, repeated tests revealed findings compatible with the diagnosis of encephalitis. Due to the suspicion of herpes simplex encephalitis the patient was treated with acyclovir. Within a few days rapid resolution of the fever and complete recovery were observed. Cytomegalovirus encephalitis should be considered early in the evaluation of pregnant women if appropriate clinical symptoms are present.

Subacute necrotizing fasciitis caused by gas-producing Staphylococcus aureus.

Presented here is a case of necrotizing fasciitis that developed bilaterally on the thighs of a 54-year-old diabetic woman following subcutaneous insulin injection. Severe localized pain was the presenting symptom; later, soft-tissue gas appeared. Incisional biopsy, performed on day 10 following admission, confirmed the diagnosis. Staphylococcus aureus was the only pathogen isolated. The disease had a slowly progressive course despite appropriate medical treatment, and recovery of the patient was achieved only after fasciotomy, drainage, and debridement of necrotic tissue was undertaken 4 weeks following admission. Staphylococcus aureus may cause subacute necrotizing fasciitis, and infection with this organism should be considered in cases of soft-tissue infection with gas formation in diabetics. The development of soft-tissue infection at the site of insulin injection should alert physicians to the possibility of infection with Staphylococcus aureus.