Pyoderma gangrenosum and seronegative erosive polyarthritis.

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