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I . Youinou P, Moutsopoulos HM, Pennec YL: Clinical features of SjBgren's syndrome. Curr Opin Rheumat01 2:687-692. 1990 2. Moutsopoulos HM, Youinou P: New developments in Sjogren's 9 1 syndrome. Curr Opin Rheumatol 3:815-822, 1 3. Chisholm DM, Mason DK: Labial salivary gland biopsy in Sjogren's syndrome. J Clin Pathol 21:6S6-660, 1968 4. Greenspan JS, Daniels TE, Talal N , Sylvester RA: The histopathology of Sjogren's syndrome in labial salivary gland biopsies. Oral Pathol 37:217-229,1974 5 . Daniels TE, Silverman S Jr, Michalski JP. Greenspan JS, Sylvester RA, Talal N : The oral component of Sjogren's syndrome. Oral Surg 39:875-885,1975 6. Daniels TE: Labial salivary gland biopsy in Sjogren's syn-

drome: assessment as a diagnostic criterion in 362 suspected cases. Arthritis Rheum 27:147-156, 1984 7. Vitali C, Tavoni A, Simi U, Marchetti G, Vigorito P, d'Ascanio A. Neri R, Cristofani R, Bombardieri S: Parotid sialography and minor salivary gland biopsy in the diagnosis of Sjogren's syndrome: a comparative study of 84 patients. J Rheumatol 15:262267. 1988 8. Lindahl G, Lefvert AK. Hedfors E: Periductal lymphocytic infiltrates in salivary glands in myasthenia gravis lacking Sjiigren's syndrome. Clin Exp lmmunol 66%-102. 19x6 9. Lindahl G . Hedfors E: Lymphocytic infiltrates and epithelial HLA-DK expression in lip salivary glands in connective tissue disease patients lacking sicca: a prospective study. Br J Kheumatol 28293-298. 1989 10. Manthorpe R, Oxholm P. Prause U. Schiodt M: The Copenhagen criteria for Sjdgren's syndrome. Scand J Kheumatol SUPPI61:19-21,1986

Decreased hemoglobin levels in nonsteroidal antiinflammatory drug-treated patients may not be related to the drug We recently reviewed the hemoglobin analyses from several well-controlled double-blind studies in which rheumatoid arthritis patients were treated either with naproxen, with a new antirheumatic drug (prinomide, CGS 10787B; Ciba-Geigy, Summit, NJ), o r with placebo. Many people believe that the use of nonsteroidal antiinflammatory drugs (NSAIDs) is frequently associated with decreases in hemoglobin levels. However, in the studies we reviewed, it was noted that some patients being treated with placebo, as well as those treated with the active drug, had decreases in their hemoglobin levels of 1-2 gm/dl over an 8-12-week period. Table 1 shows the number of patients in each treatment group who had significant decreases (1-2 gm/dl) in their hemoglobin values. As seen, 13.7% of the placebo-treated patients had a significant decrease over a relatively short period of time (4-12 weeks of treatment), compared with 6 2 2 % in the drug-treated groups. Since reduction in the hemoglobin level is a common finding in patients with rheumatic diseases. it is reasonable to infer that an improvement in disease symptoms may be reflected by an increase in the hemoglobin level and, conversely, that a decrease in hemoglobin level accompanies a flare of symptoms. In an effort t o determine whether there

Table 1. Patients with decreases of 1-2 g d d l in the hemoglobin (Hgb) level. by treatment group

No. (R) of

patients

Mean 2 SD decrease, gddl

with de-

creased Hgb

Treatment (n) ~~~~~~~~

~~~

Range of decreases, gddl

~

Prinomide. 1,500 &day (86) 18 (20.9) 1.42 2 0.26 Prinomide, 1,(K)0mg/day (46) 4 (8.7) I.23 2 0.32 3 (6.0) 1.42 2 0.46 Prinomide. 500 mg/day (50) Naproxen. 750 mg/day (90) 20 (22.2) 1.3 2 0.27 18 (13.7) 1.33 2 0.24 Placebo ( I3 I )

1.0-1.7 1.&I.9 I.O-2.0 I.%I .9

1.0-1.8

was ;I relationship between the decreases in hemoglobin values and symptomatic changes in these patients. correlations between decreases in hemoglobin level and changes in 4 major efficacy parameters were determined. The efficacy parameters chosen were investigator and patient assessment of disease severity (measured on a 10-cm visual analog scale), change in the number of tender joints (maximum 68). and change in the number of swollen joints (maximum 66). Using the Pearson product-moment method, no correlation was found between decreases in hemoglobin level and changes in any of these parameters. The amount of blood drawn over the 8-12-week period of these studies (150-200 ml per patient) would not normally be expected to induce a 1-2 gm/dl decrease in the hemoglobin level. These data indicate that significant decreases in the hemoglobin level are not necessarily related to either NSAlD use o r disease state, since nearly 14% of the placebo-treated patients had significant decreases in their hemoglobin levels, and there was no correlation with disease activity.

D. Innes Cargill, PhD Tenere Consirlting Tarrytown, N Y Daniel E. Furst, M D Robert Wood Johnson Medical School N e w Brunswick, NJ

Spinal cord compression by epidural lipomatosis in a patient with systemic lupus erythematosus Epidural lipomatosis, a rare complication of systemic glucocorticoid therapy, is characterized by abnormal accumulations of unencapsulated white fat on o r outside the dura. Unlike excessive fat accumulations in other areas, excessive deposition of fat in the epidural space may cause serious morbidity, including complete paraplegia. In 1975, Lee e t al reported the first case of epidural lipomatosis in a renal transplant patient ( I ) . In other patients with this

CONCISE COMMUNICATIONS condition, reported underlying medical problems have included heart transplant, polyarteritis nodosa, asthma, Graves’ disease, lymphoma, hepatitis, rheumatoid arthritis, juvenile rheumatoid arthritis, and dermatomyositis (2). Epidural lipomatosis has not previously been described as a complication of systemic lupus erythematosus (SLE). We describe herein an S L E patient in whom this condition developed. The patient was a 28-year-old, morbidly obese woman with a 10-year history of steroid-dependent SLE. Manifestations of lupus included a generalized seizure disorder, arthritis, facial rash, Raynaud’s phenomenon, myositis, pleurisy, peripheral neuropathy, and an affective disorder characterized by depression. She had been taking prednisone at moderately high doses (>30 mg/day) for more than 10 years. Immunotherapy with azathioprine and methotrexate had been initiated but was discontinued due to lack of efficacy a n d o r toxicity. Four to five months prior to admission, the patient developed back pain. One week prior to our evaluation, she suddenly developed bilateral leg weakness and urinary and fecal incontinence. Her prednisone dosage at the time of admission was 40 mg/day. Examination revealed an obese woman with cushingoid features. Results of a general physical examination

483 were unremarkable except for the obesity and striae. Neurologic findings were normal except in her legs. Motor strength testing revealed 115 strength in the right hip flexors and 215 on the left. The knee flexor and extensor strength was 015 bilaterally. Ankle dorsiflexion and plantar flexion strength was 015 on the right and 115 and 215, respectively, on the left. Sensation to pin prick and light touch were diminished below the level of T8. Proprioception, vibratory sense, and deep tendon reflexes were absent in the legs. The rectal examination revealed flaccid tone. Abnormal laboratory data included a white blood cell count of 28,000/mm3, a Westergren erythrocyte sedimentation rate of 41 m d h o u r , and gamma glutamyl transferase and lactate dehydrogenase levels of 569 unitslliter and 301 unitslliter, respectively. An anteroposterior radiograph of her thoracic spine revealed a possible T5 fracture. Magnetic resonance imaging of the thoracic and lumbar spine demonstrated multiple old compression fractures of the lumbar vertebral bodies and a subacute compression fracture of TS, with associated hematoma (Figure I). There was excessive epidural fat present in both the lumbar and thoracic regions (Figure 1). The epidural fat in the thoracic region occupied more than 50% of the spinal

B Figure 1. A, Sagittal and B, axial images of the thoracolumbar spine. Curved (white) arrows show subacute compression fracture of T5 with associated hemorrhage. Straight (black) arrows show extensive intraspinal fat. The hemorrhage, fracture, and epidural fat produce ventral compromise of the thecal sac.

A

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484 canal. causing significant compromise of the thecal sac. A diagnosis of spinal epidural lipomatosis was made. It was believed that the compression fracture, hematoma, and lipomatosis were compressing the spinal cord at T5. A neurosurgeon was consulted about possible spinal cord decompression. Because of the long duration of symptoms, morbid obesity, extensive length of the fat deposit, and the need for internal fixation, it was concluded that the operative risks were prohibitive in this patient. Strict dietary control and reduction of the steroid dosage were recommended. Azathioprine was reinstituted at a dosage of 75 mg once daily, and the prednisone dosage was decreased to 30 mg/day. After 6 months of intensive physical therapy, during which time the prednisone dosage was further reduced to 17.5 mg/day, muscle strength testing revealed 2/5 strength in the right hip flexors and 315 on the left. Knee extension was 4 6 bilaterally. Foot extensor strength was 4/5 on the right and 515 on the left. The patient's weight had decreased to 230 pounds. She still had urinary incontinence but had bowel control. Epidural lipomatosis, a condition characterized by abnormal accumulations of unencapsulated fat on or outside the dura, is extremely rare. This medical complication of regular steroid use is unpredictable, but appears to be related more to the duration of steroid administration than to dosage. The presence of excessive fat in the spinal canal may compromise the thecal sac to such an extent that a relatively minor insult, such as the compression fracture in the present case. causes symptoms. Fat deposition occurs most commonly in the thoracic region, but involvement of the lumbar and sacral areas has been described. This diagnosis should be considered in any patient receiving corticosteroids who presents with signs or symptoms of spinal cord or cauda equina compression. Differential diagnoses of these signs and symptoms include vertebral fractures, hematoma, epidural abscess, transverse myelitis, arteriovenous malformation, congenital spinal stenosis, and malignancy, any of which may be present in addition to epidural lipomatosis (2). Myelography, though sensitive, cannot be used to make a specific diagnosis. The diagnosis is best made by computed tomography because fat has a low specific density (-80 to -120 Hounsfield units) (3). Surgical decompression is often indicated for treatment of epidural lipomatosis, and it can result in either rapid relief of symptoms or slow recovery over months. Immediate decompressive laminectomy should be considered if there is severe, acute compression with progressive neurologic signs. Conservative treatment with steroid reduction and dietary restriction for weight loss have been variably successful, especially in patients whose symptoms are mild or whose general condition precludes surgical intervention (4). Hulon E. Crayton, MD Curtis R. Partington, MD Carolyn L. Bell, MD University of Wisconsin Hospital and Clinics Modison, WI

I. Lee M, Lekias J , Gubbay SS, Hurst PE: Spinal cord compression by extradural Fat after renal transplantation. Med J Ausl 1201203, 1975 2. Chapman PH, Martuza KL. Poletti CE, Karchmer AW: Lipomatosis associated with Cushing's syndrome. Neurosurgery 8:724727, 1981 3. Haid RW Jr, Kaufman HH. Schochet SS, Marano GD: Epidural lipomatosis simulating an epidural abscess: case report and literature review. Neurosurgery 2 I :744-747, 1987 4. George WE, Wilmot M. Greenhouse A, Hammeke M: Medical management of steroid induced epidural lipomatosis. N Engl J Med 308:31&319, 1983

Sweet's syndrome and monarthritis in a human immunodeficiency virus-positive patient

Sweet's syndrome is an acute febrile neutrophilic dermatosis, mostly affecting young adults ( I ) . It can be associated with hematologic disorders, such as myeloid or myelomonocytic leukemia. The exact etiology of Sweet's syndrome is unknown, but a n infectious origin has been suggested. We present herein the first report of Sweet's syndrome occurring in association with human immunodeficiency virus (HIV) infection. The patient also had a monarthritis that was not typical of the arthritis often seen in patients with Sweet's syndrome. The development of Sweet's syndrome could be related to hypersensitivity to a viral antigen. The patient, a 30-year-old nurse, was admitted to the hospital in July 1988 for evaluation of persistent swelling of the left knee. Her clinical symptoms had begun 1 month earlier, with the sudden onset of red lesions on the skin of the face and the dorsal aspects of the hands and the wrists. Sweet's syndrome was diagnosed on the basis of the clinical findings. Prednisone (30 mg/day) was begun, and the symptoms improved rapidly. Ten days after admission, new skin plaques appeared, and the patient reported having pain and swelling of the left knee. Her prednisone dosage at that time was 10 mg/day. The patient had a temperature of 38.2"C. and swelling of the axillary lymph nodes was seen on physical examination. A skin biopsy revealed perivascular and diffuse dermal infiltration by polymorphonuclear cells, associated with mild leukocytoclastic vasculitis (Figure 1). The white blood cell count was 3.4 x 1Oy/liter,with 1,598 polymorphonuclear cells (PMN)/mm3 and 1,666 lymphocytes/mm'; the erythrocyte sedimentation rate was 62 mm/hour. Rheumatoid factor and antinuclear antibodies were not found; anticardiolipin antibodies were detected by enzyme-linked immunosorbent assay (ELISA; titer > I :200). Tests for circulating immune complexes (polyethylene glycol-Clq) gave positive results. HIV was detected in the serum by ELISA. and Western blot analysis showed antibodies to gp160, gp141, pcore55, pcore40, ppo168, and ppo134. The CD4 lymphocyte count was low (283/mm3); the CD4:CD8 ratio was 0.25. HIV was not found in white cells