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Extensive phalangeal cystic lesions.

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123
EXTENSIVE PHALANGEAL
CYSTIC LESIONS
SARCOIDOSIS LIMITED T O T H E HANDS AND FEET?
R. A. SCHRIBER and H. FIROOZNIA
A young black man is presented with a 33-month
course of insidious but eventually striking hand deformity and limitation of motion in the absence of any
other symptoms. Radiologic and histologic findings of
the digits support the diagnosis of sarcoidosis, though
physical, laboratory, and chest x-ray examination show
no evidence of this multisystem disease.
Osseous involvement is a well-recognized feature
of sarcoidosis, occurring rarely in the absence of skin
lesions or chronic, multiorgan system involvement.
When present it is usually not symptomatic. A patient
was presented with complaints limited to the hands
and feet throughout a 33-month course. The possibility
is raised that, in rare instances, sarcoid may be limited
to the skeletal system.
CASE REPORT
A 19-year-old black man was admitted to Bellevue
Hospital for the first time in September 1972 for hand swellThis work was supported in part by the National Institutes of Health Training Grant in Arthritis AM 0 5064.
Robert A. Schriber, M.D., Fellow, Rheumatic Diseases
Study Group, New York University School of Medicine; Hossien
Firooznia, M.D., Assistant Professor of Radiology, New York University School of Medicine.
Address reprint requests to Robert A. Schriber, M.D.,
c/o Dr. Zudter-Franklin, Room H-445, New York University
Medical Center, 550 First Avenue, New York, New York 10016.
Submitted for publication March 21, 1974; accepted June
11. 1974.
Arthritis and Rheumatism, Vol. 18, No. 2 (March-April 197.5)
ing and limited motion of both hands of 19 months duration.
The patient was well until January 1971 when he
noted swelling and mild discomfort in the area of the second left metacarpophalangeal joint, which lasted several
days and resolved. This condition recurred soon afterward
in several areas of both hands. At all times, swelling was
the predominant feature, although there was mild discomfort at times, especially in the morning. He came to the
outpatient department at Bellevue Hospital in early 1971
for this complaint. Radiographic examination of the hands
showed several cystic bony lesions, but the patient did not
return for further workup or treatment.
During the next 19 months he noted persistent swelling of the fingers with occasional mild discomfort and morning stiffness. However, he was not greatly bothered and
continued his activities as a student, even playing basketball
occasionally. He sought no medical attention during this
time. I n the summer of 1972, the patient noted that the
swelling increased and that he now had difficulty performing certain fine movements because of relatively painless
limited motion. When school started in September 1972, he
noted he could not hold a pencil properly and sought
medical attention for the second time at Bellevue Hospital.
Pain was never a major feature and there were no acute
at tacks.
There was no history of fever, malaise, rash, other
musculoskeletal complaints, visual disturbances, cough, or
shortness of breath. Past medical history and family history
were noncontributory. On admission to Bellevue Hospital
in September 1972, physical examination revealed a wellnourished, well-developed black man who appeared healthy,
except for obviously swollen, sausage-like fingers. Vital signs,
head, eyes, ears, nose, and throat were normal. There were
no skin lesions. There were several y2 X
cm soft, movable, nontender nodes in both axillae. The chest cage, heart,
and lungs were normal. The abdomen showed no organo-
v2
SCHRIBER AND FIROOZNIA
124
Fig 1. Photograph of patient’s hands, September 1972.
megaly. There was massive swelling of the fingers bilaterally.
T h e wrists and metacarpal phalangeal joints were normal,
but distal to the metacarpal phalangeal joints there was
marked soft boggy swelling that seemed to involve both the
joints and the phalanges. T h e lesions were nontender and
the overlying skin was normal (Figure 1). There was marked,
limited motion of the interphalangeal joints. There were
no nodules, tophi, or other joint involvement. Two interphalangeal joints of the left foot appeared swollen. T h e
neurologic examination was normal. X-rays of the hands
showed very extensive reticular and cystic destructive lesions
involving most of the phalanges of both hands. There was
marked soft tissue swelling of both hands and narrowing
of the proximal interphalangeal joints (Figure 2). T h e feet
showed similar though less marked changes. Compared to
1970, the x-rays showed marked worsening. Complete blood
count, urinalysis, blood urea nitrogen, creatinine, glucose,
uric acid, calcium, phosphorous, liver functions, including
scan and alkaline phosphatase, liver functions, including
VDRL, latex agglutination, and electrocardiogram were all
normal. T h e chest x-rays showed normal lung fields and
hila (Figure 3). T h e patient was anergic to the following
antigens: intermediate PPD, trichophyton, candida, and
SK/SD. Sensitization with dinitrochlorobenzene was attempted and was unsuccessful. Finally an open biopsy of
the finger was performed, revealing noncaseating granulometa negative for acid-fast bacilli and fungi (Figure 4).
Twenty-four-hour urine calcium, pulmonary function, and
in vitro lymphocyte studies were not performed. The Kveim
test was negative but was performed at a time when steroids
had already been started.
T h e patient was started on 30 mg of prednisone in
September 1972. Over the next 3 months, the swelling decreased about 3070-50% and the range of motion improved
dramatically. Repeat x-rays in January 1973 showed definite
but early filling in of lesions. At present the patient is on
15 mg of prednisone and has complete use of his hands,
with excellent range of motion and grip strength though
the sausage-shaped swelling is still quite obvious. X-rays
taken in January 1974 show marked improvement (Figure 5).
DISCUSSION
Sarcoidosis is generally considered a multisystem
granulomatous disease of unknown etiology. T h e patient presented the following features: a) 18 months of
osseous disease of the hands and feet with increasing
deformity and limitation, but minimal pain; b) anergy; c) striking radiologic lesions; d) noncaseating
granulomas in the affected bone. T h e diagnosis of
sarcoidosis limited to the bone is compelling in this
case, although some authorities require the presence
EXTENSIVE PHALANGEAL CYSTIC LESIONS
Fig 2. Radiograph
of hands, September 1972.
Pig 3. Radiograph of chest (PA and lateral), July 1972. T h e lung fields and mediastinum are normal.
126
Fig 4. Photomicrograph of finger biopsy, September 1972.
of multiorgan involvement to make this clinical diag-
nosis with certainty. Liver, scalene node, or muscle
biopsy might have provided additional evidence but
were considered unwarranted in view of the normal
chest x-ray, liver function tests (including alkaline
phosphatase), and the absence of musculoskeletal complaints other than limitation of motion. The characteristic histologic findings and the highly typical radiologic picture support the diagnosis of sarcoidosis. In
the differential diagnosis of such a radiologic picture,
hyperparathyroidism, polyostotic fibrous dysplasia, and
multiple enchondromata could be considered. However the absence of other characteristic features and
the histologic findings rule these out. Rheumatoid arthritis and gout can produce cystic lesions similar to
sarcoid, but not the diffuse, reticular lattice-work pat-
SCHRIBER AND FIROOZNIA
tern with thinning and expansion of the cortex seen
in this patient. Also excluding these illnesses are the
minimal amount of pain, the histologic picture, the
negative latex fixation tests, and the normal uric acid.
Tuberculosis and fungal dactylitis nearly always demonstrate extensive periostial new bone formation at
the site of the destructive lesions. The special stains
and cultures of the biopsy specimens were negative in
this regard.
Musculoskeletal sarcoidosis seems to present two
separate and, for the most part, contrasting clinical
pictures: a) osseous sarcoid and, b) sarcoid arthritis.
Osseous sarcoid was first described by Kreibach
in 1904 (l), and in 1928 Jungling (2) described the
highly characteristic radiologic lesions, similar to more
recent series (3,4). Any bone can be involved, but the
hands and feet are by far the most common sites. The
incidence of osseous lesions varies from 30% to less
than 20/, in most series. Series with a high prevalence
of skin sarcoids or those with a heavy representation
of chronic cases with .multiorgan involvement tend
toward the higher incidence figures (3-8). The low incidence series (< 5y0) (9-12) are weighted with early
cases, especially with bilateral hilar adenopathy, erythema nodosum, and a low incidence of skin lesions.
The clinical presentation of osseous sarcoid is most
remarkable by its absence of symptoms in most cases.
Many are discovered by x-ray only. Even with obvious
deformity, pain is usually minimal (3,6,8,10). Osseous
lesions have never been specifically noted in the literature as the sole manifestation of sarcoidosis, although
some early European reports (1,2) imply this possibility in a few cases prior to the routine use of chest
radiography.
On the other hand, many sarcoid patients have
prominent musculoskeletal symptoms, frequently a relatively acute polyarthritis or arthralgia for the most
part involving' larger joints. This symptom occurred in
nearly 20% of an unselected sarcoid clinic population
at Johns Hopkins (13). As in other descriptions of the
syndrome (9,14,15,IS), early cases with fever, bilateral
hilar lymphadenopathy, erythema nodosum, and peripheral lymphadenopathy were predominant. Biopsy
showed noncaseating granuloma in the synovium of
half of Bianchi and Keech's cases (14) and in Myers'
cases (15). Osseous lesions were remarkably absent (15,
16) or rare (1 3,14).
Sokoloff and Bunim (17) reported 5 cases of a
more chronic polyarthritis in five young blacks. Four
had noncaseating granulomas on synovial biopsy.
127
EXTENSIVE PHALANGEAL CYSTIC LESIONS
Fig 5. Radiograph of hands, January 1974. There is striking
improvement compared to radiograms of Septeiuber 1972, with
mcrch sclerotic filling in of the lesions.
Their sarcoid was multiorgan in all cases (2 skin, 1
uveitis, 4 pulmonary, and 4 hilar nodes), and noncaseating granulomas were sought and found in another
tissue besides the synovium in all cases (3 lymph nodes,
1 muscle, 2 skin, and 1 liver). Typical osseous lesions
were seen in 3 and joint destruction in 1. Similarly,
Spilberg et a1 included 4 cases in their series (18),
which they classified as chronic sarcoid arthritis. All
had lung involvement, 3 had skin, 3 nodal, and 2
had ocular involvement. Joint erosions were present
in 2 by x-ray, but only 1 had phalangeal cystic lesions
and these were minimal.
T h e type of bone involvement in these patients
probably comes closer to that seen in our patient, although he is quite unusual in his absence of obvious
organ involvement other than musculoskeletal.
REFERENCES
1. Kreibach K: Uber lupus pernio. Arch Dermatol Syphilol
(Wien) 71:3, 1904
2. Jungling 0: Uber ostitis tuberculosa multiplex cystoides.
Beitr Klin Chirurg 143:401, 1928
3. Holt JF, Owens WI: The osseous lesions of sarcoidosis.
Radiology 53: 11, 1949
4. Israel H, Sones D: A roentgenographic study of skeletal
lesions in sarcoidosis. Arch Intern Med 102:766, 1958
5. Gravense PB: Lymphogranulomatosis Benigna. Odense,
1942
6. Longcope WT, Frieman DG: Study of sarcoidosis. Medicine 31:1, 1952
7. Gilg I: Kliniske undersogelser over Boecks sarcoid.
Copenhagen, 1955
8. Reisner D: Boeck’s sarcoid and systemic sarcoidosis.
Ann Rev Tuberc 49:299, 1944
9. Lofgren S: Primary pulmonary sarcoidosis. Acta Med
Scand 145: 465, 1953
10. Mather G: Calcium metabolism and bone changes in
sarcoidosis. Br Med J 1:248, 1957
11. James DG: Dermatological aspects of sarcoidosis. Q J
Med 28: 109, 1959
12. Scadding JG: Sarcoidosis. London, Eyre and Spottiswoode, 1967, pp 208-227
128
13. Gumpel R, Shulman I: T h e joint disease of sarcoidosis
Ann Rheum Dis 26:194, 1967
14. Bianchi FB, Keech MK: Sarcoidosis with arthritis. Ann
Rheum Dis 23:463, 1964
15. Myers GB: Joint and skeletal muscle manifestations in
sarcoidosis. Am J Med 12:161, 1952
16. Caplan A: Periarticular inflammation, bilateral hilar
SCHRIBER AND FIROOZNIA
adenopathy and a sarcoid reaction. Arthritis Rheum 1.3:
101, 1970
17. Sokoloff L, Bunim JJ: Clinical and pathological studies
of joint involvement in sarcoidosis. N Engl J Med 260:
841, 1959
18. Spilberg I, Siltzbach L, McEwen C: T h e arthritis of
sarcoidosis. Arthritis Rheum 12: 126, 1969
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