LETTERS Pyuderma gangrenosum and seronegative erosive pdyarthritis To the Editor: Pyoderma gangrenosum was initially described in 1930 (1) in 5 patients, 4 of whom had ulcerative colitis. Up to 50% of cases of pyoderma gangrenosum have since been recognized as occurring with ulcerative colitis; Crohn’s disease, myeloproliferative disorders, and paraproteinemias are more recently accepted associations. An estimated 30% of patients with pyoderma gangrenosum suffer joint s y m p toms (2), but no single pattern of arthritis has consistently emerged. Evidence is accumulating, however, for a relationship between pyoderma gangrenosum and seronegative erosive polyarthritis. We present a patient with such a combination. A 54-year-old white woman with a 15-year history of erosive seronegative polyarthritis was admitted to the hospital with 2 painful ulcers characteristic of pyoderma gangrenosum on the lateral aspect of the left thigh. Examination revealed bilateral, destructive arthritis particularly involving the metacarpophalangeal joints, elbows, hips, knees, and subtaloid joints with fixed flexion deformities of the elbows, hips, and knees. There were no eye, bowel, skin, or genitourinary symptoms or signs and no relevant family history. Investigations confirmed negative sheep cell agglutination titer. Radiologic examination of the affected joints showed erosive disease with involvment of the odontoid peg and subluxation at Cl-C2 and C4-CS levels. Biopsies from an ulcer edge showed nonspecific inflammatory changes with no evidence of vasculitis. The ulcers increased in size, and a treatment regimen of prednisolone with azathioprine was commenced. Within 48 hours of beginning treatment, the patient developed septicemia with multiple joint sepsis. She died as a result, despite full antibacterial and supportive treatment. Aspirates from the left elbow and left knee, venous blood, and swabs from the ulcers all cultured the same Lancefield groups C phemolytic streptococcus. Request for autopsy was refused. Although a relationship between pyoderma gangrenosum and joint disease is generally accepted, a review of the literature shows 3 main types of arthropathy being identified. First, the characteristic arthritis of ulcerative colitis occurs. This is normally an acute, nonerosive, oligoarthritis of the lower limbs. Wright and Watkinson (3) estimated this to occur in 11.5% of patients with ulcerative colitis. These researchers showed a relationship between arthropathy and disease activity in the gut and, particularly in women, with the degree of colonic involvement. The early studies of pyoderma gangrenosum in ulcerative colitis ( 4 3 suggested similarly that the skin lesions were associated with the severity of the inflammatory bowel disease. Later reports, however, did not confirm this direct relationship ( 6 8 ) . It can be argued, therefore, that both skin and joint complications are related directly to the bowel 813 disorder and, as a corollary, that no direct relationship between joint and skin disease has been shown to exist. Second, an association between pyoderma gangrenosum and classic rheumatoid arthritis (RA) has been suggested. In 1958 (8) 4 cases of arthritis and pyoderma gangrenosum were documented. Although confirmatory laboratory test findings were unavailable, 3 of these patients had a distribution of arthritis compatible with RA. Lazarus and colleagues in 1972 (9) described 3 similar cases. In these, however, tests for rheumatoid factor were negative and 2 of the patients had nonerosive disease (although in the third a rheumatoid nodule and erosive changes were present and test for antinuclear factor positive). The two patients in a 1975 report by Stolman et al (10) were seropositive. Although clinically the skin lesions were characteristic of pyoderma gangrenosum, biopsies of these revealed necrotizing vasculitis. More convincingly, a review article by Hickman and Lazarus (11) showed an association in 3 patients between pyoderma gangrenosum and seropositive classic RA, in which vasculitis had been excluded on biopsy. The anecdotal nature of all these reports unfortunately precludes an unequivocal conclusion that a relationship exists between pyoderma gangrenosum and RA, as the latter is not an uncommon disease. The difficulty of joint disease classification in pyoderma gangrenosum is highlighted in a large series of patients in Great Britain, described by Holt et a1 in 1980 (12). Eight patients with inflammatory polyarthritis of a total of I5 patients with pyoderma gangrenosum were reported. Two of these had seropositive RA, 1 seronegative polyarthritis in conjunction with chondrocalcinosis, and 1 seronegative polyarthritis with ulcerative colitis. The 4 other patients had polyarthritis of the type frequently accepted as showing a definite relationship to pyoderma gangrenosum. This third group had seronegative, erosive polyarthritis occumng without evidence of other disorders. Holt and coauthors present a cogent hypothesis for the presence of a distinctive seronegative arthropathy associated with pyoderma gangrenosum. The joint disease in 3 of their 4 patients antedated the skin lesions, and its distribution in all 4 was unlike that seen with the spondylarthritides. We believe that strong evidence exists only for the thud combination described-pyoderma gangrenosum with a particular, seronegative, destructive arthritis. We have presented a patient with many features similar to those of Holt’s patients, in support of this. Thomas Palferman, MB, MRCP Graham Colver, MB, MRCP David Doyle, MD, MRCPI Ian Wright, MB, BS Stephen Ebbs, MB, BS Whipps Cross Hospital London, England 8 14 1. Brunsting LA, Goeckerman WH, O’Leary PA: Pyoderma (ecthyma) gangrenosum: clinical and experimental observations in five cases occurring in adults. Arch Dermatol 22:655-680, 1930 2. Van der Sluis I: Two cases of pyoderma (ecthyma) gangrenosum associated with the presence of abnormal serum protein (b2 A-paraprotein). Dermatologica 132:409-424, 1966 3. Wright V, Watkinson G: The arthritis of ulcerative colitis. Br Med J 3:670-675, 1%5 4. Rice Oxley JM, Truelove SC: Complications of ulcerative colitis. Lancet 1:607-611, 1950 5 . Watts JMcK, De Dombal FT, Watkinson G. Goligher JC: Long term prognosis of ulcerative colitis. Br Med J 1: 1447-1453, 1966 6. Johnson ML, Wilson MTH: Skin lesions in ulcerative colitis. Gut 10~255-263, 1969 7. Perry HO: Pyoderma gangrenosum. South Med J 62:899-908, 1969 8 . Ayres S Jr, Ayres S 111: Pyoderma gangrenosum with an unusual syndrome of ulcers, vesicles and arthritis. Arch Dermato1 77~269-280, 1958 9. Lazarus GS, Goldsmith LA, Rocklin RE, Pinals RS, de Buisseret JP, David JR, Draper W: Pyoderma gangrenosum, altered delayed hypersensitivity, and polyarthritis. Arch Dermatol 105:46-51, 1972 10. Stolman LP, Rosenthal D, Yaworsky R, Horan F: Pyoderma gangrenosum and rheumatoid arthritis. Arch Dermatol 111: 1020-1023, 1975 11. Hickman JG, Lazarus GS: Dermatology Update. 1979 Edition. New York, Elsevier, 1979, pp 325-342 12. Holt P, Davies MG, Saunders KC, Nuki G: Pyoderma gangrenosum: clinical and laboratory findings in 15 patients with special reference to polyarthritis. Medicine (Baltimore) 59: 114-133, 1980 Rheumatoid synovial cyst of the hip To the Editor: In a recent issue of Arthritis and Rheumatism, Levy et al (1) described a patient with rheumatoid arthritis who developed a synovial cyst of the left hip; they reported this to be a rare disorder. We would like to add a report of a 56year-old white woman with classic rheumatoid arthritis, functional class 4 ( 2 ) , of 25 years’ duration, treated with low dose corticosteroids. She was admitted to the rheumatology unit of De Wever Hospital in December 1981 because of pain and intermittent swelling of the left leg. Physical examination on admission revealed a swelling in the left lower quadrant of the abdomen, extending below the inguinal ligament downwards into the left thigh. Roentgenogram of the pelvis showed destruction of both hip joints, with the joint space hardly recognizable and the femoral head extensively destroyed with signs of avascular necrosis. Computed tomography (CT) revealed a cystic process anterior to the left iliopsoas muscle, extending from the fourth lumbar vertebra to the upper half of the left thigh. LETTERS Figure 1. Contrast enhanced computed tomography scanning of the pelvis. A fluid-filled cavity is seen, likely arising from the joint space of the left hip joint. One picture suggested a connection between this process and the articular space of the left hip joint (Figure 1). For further diagnosis a limited surgical exploration was performed through a suprailiac incision. During this procedure a large extraperitoneal cyst was drained and a biopsy specimen of the wall was taken. Microscopic examination of the biopsy specimen showed focal necrosis and fibrin deposition, with palisaded cells vertical to the margin of the necrosis, surrounded by a lymphocellular infiltrate suggestive of a “rheumatic nodule.” Conventional aerobic and anaerobic bacteriologic cultures were sterile, and cultures for tubercle bacilli were negative. Radical surgical removal of the cyst was not possible because of the patient’s poor general condition. A continuous drainage system was left for 3 weeks, until no more filling of the cyst could be demonstrated clinically o r by CT scan. Recent control scanning, however, has shown a gradual accumulation of fluid, although it is not yet clinically demonstrable. In conclusion, this patient with destructive rheumatoid hip disease had an extensive iliopectineal cyst, obviously communicating with the remnants of the left hip synovial cavity, demonstrated by computed tomography and surgical exploration. Computed tomography seems to be an adequate noninvasive method for diagnostic imaging of this relatively rare condition (3). Piet Jacobs, MD Henk S. Goei The, MD Augustus Bijlsma, MD Cees W. Versteege, MD De Wever Hospital Heerlen, The Netherlands
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