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Sternocostoclavicular hyperostosiscase report with a 31-year followup.

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Sternocostoclavicular h yperostosis (SCCH),
also called intersternocostoclavicularossification and,
more recently, pustulotic arthroosteitis, is a rare syndrome in western countries. However this interesting
osteoarticular disease, often associated with a palmoplantar pustulosis, seems to occur frequently in Japan.
The case described here, one of the very few in
Europe and North America, is notable in that it was
possible to follow the patient’s course over 31 years.
Case report. The patient, a 51-year-old male
radio-television technician, reported having had sternoclavicular pains with progressive stiffening of the
shoulder girdle since he was 20 years old. When he
was serving in the army, he had had difficulty in
flinging objects normally and aligning his rifle in the
“shoulder arms” position. Radiographs taken when he
was 25 years old showed normal sternoclavicular
joints but signs of a manubriosternal erosive arthritis.
During the next 20 years the disease was characterized by 2 essential features: occasional bouts of
inflammatory pain localized at the sternocostoclavicular junction (without swelling or redness of the soft
tissues) and a total bilateral stiffness of the shoulder
girdle. The pains were clearly aggravated by cold and
alcohol consumption and did not respond to nonsteroidal antiinflammatory drugs.
From the Department of Medicine, Division of Rheumatology, Geneva University Medical School, Geneva, Switzerland.
Georges H. Fallet, MD: Professor of Rheumatology; Jesus
Arroyo, MD: Resident; Thomas L. Vischer, MD: Assistant Professor of Rheurnatology, University Hospital, Geneva, Switzerland.
Address reprint requests to Dr. G. H. Fallet, Division of
Rheumatology, University Hospital, CH-1211 Geneva 4, Switzerland.
Submitted for publication July 21, 1982; accepted in revised
form November 11, 1982.
Arthritis and Rheumatism, Vol. 26, No. 6 (June 1983)
When the patient was 44 years old, a medical
examination revealed the following: erythematosquamous skin lesions at both elbows, clinically and histologically typical of psoriasis vulgaris (but with no
palmoplantar pustulosis or lesions of the nails) (Figure
I); a marked atrophy of all the muscles of the shoulder
girdle, which was completely stiffened; roentgenograms showing a complete bone fusion, joining the
clavicles to the sternum and to the first ribs symmetrically on both sides (Figure 2); wmtechnetium pyrophosphate bone scan showing an increased uptake of
the sternocostoclavicularjunction; asymptomatic erosive lesions of the upper and lower endplates of C5 and
lower endplate of C6 with osteosclerotic remodeling of
the C5 vertebral body (Figure 3); and all laboratory
investigations normal except for an erythrocyte sedimentation rate (Westergren) of 35 mdhour.
The patient noticed that his pains subsided
beginning when he was age 44, and they eventually
disappeared from the upper sternal region; they moved
more distally along the two clavicles where they
alternated from one side to the other. The sensitivity
to cold had disappeared. Three years ago, a fall from a
motorcycle resulted in a fracture of the left clavicle,
which healed quickly as a result of simple conservative
The same year the patient developed, suddenly
and for the first time, vesiculopustular lesions localized to the soles and lateral aspects of both feet. These
lesions have persisted ever since.
The most recent examination in FebruaryMarch 1982 showed a patient who was generally in
excellent condition and hindered very little in his daily
and professional activities. The following findings
were, however, of note: the absence of signs of
Figure 1. Psoriatic lesion of the right elbow.
inflammation in the sternocostoclavicular region;
marked increase of the local osteosclerosis of the
sternocostoclavicular junction and of the sclerotic
hyperostosis of both clavicles, shown radiologically
(Figure 2); wmtechnetiumbone scintigraphy still showing increased uptake of the upper sternocostal area
and of the two clavicles; a slight painless stiffening of
the cervical spine, with mobility of the dorsolumbar
segment maintained and thoracic expansion of 5 cm;
comparative roentgenograms of the cervical spine
disclosing an increase of the osteosclerotic remodeling
of C5 and C6, a partial obliteration of some erosions,
and most notably, a solid bony fusion of the vertebral
bodies from C3 to C6 (Figures 3b and 4); clinical and
radiologic integrity of the limb and sacroiliac joints
(Figure 5 ) ; persistence of the psoriatic lesions on both
elbows, and the presence of a bilateral plantar pustulosis of spongiform type on histologic examination (Figure 6); normal results of laboratory evaluations including blood calcium and urate (however the erythrocyte
sedimentation rate remained slightly elevated at
25 mmhour); tests for rheumatoid factors always
negative; typing for histocompatibility antigens (HLA)
positive for A2, A31, B27, B22, Bw4, Bw6, Cwl, Cw3,
A biopsy of the right sternocostoclavicular
junction was performed. It consisted of samples of the
Figure 2. Standard radiographs of the shoulder girdle (anteroposterior view). a, 1975: developing of a bone fusion between
the clavicles, the two first ribs, and the sternum. Note that apart from the “fluffy periostitis.” the clavicles have a normal
radiologic appearance. b, 1982: complete bone fusion of the sternocostoclavicular area. The local osteosclerosis has
increased and both clavicles have considerably enlarged and become more dense.
Figure 3. Tomograms of the lower cervical spine (anteroposterior view). a, 1975: erosive lesions of the upper and lower
endplates of C5 and lower endplate of C6; osteosclerotic remodeling of C5. b, 1982: partial obliteration of the erosive
lesions and solid bony fusion of the vertebral bodies from C3 to C6.
fibrous tissue adhering to the sternoclavicular bridge;
the heterotopic bone in the form of a bridge on the
right side; and a bony fragment of the anterior aspect
of the medial third of the right clavicle. No microorganism grew on culture from the tissue specimens
taken. Histopathologic examination excluded any infectious or tumoral processes. The first sample did not
reveal anything pathologic. The costoclavicular bony
bridge was made up of a compact and cancellous
lamellar bone of slightly remodeled structure. The
clavicular sample taken showed a haversian structure
bordered by a newly formed bone intermingled with
perforating ligament bundles (Sharpey’s fibers type).
The microradiographic examination showed that the
cortex of the clavicle, particularly in the region of
ligament insertion, presented irregularities of osteon
calcification, suggesting a remodeling which was clearly more active and more recent than that in the bridge
(Figure 7).
This patient was diagnosed as having sternocostoclavicular hyperostosis (intersternocostoclavicular
ossification or pustulotic arthroosteitis). The first clini-
cal symptoms appeared at the age of 20 years, and the
first radiologic lesions were discovered at the age of 25
years. With time, the condition resulted in a complete
fusion between the two clavicles, the two first ribs,
and the sternum. It was further complicated by erosive
lesions of the cervical spine, presently showing a bony
ankylosis from C3 to C6. Furthermore, the osteoarticular process is associated with a psoriasis of both
elbows and, 23 years after its first appearance, with a
bilateral plantar pustulosis. The patient, who has no
sacroiliitis or typical syndesmophytes, carries, however, the HLA-B27 antigen but not HLA-B13, B17, or
Discussion. Since the first description of the
sternocostoclavicular hyperostosis syndrome by Sonozaki et a1 in 1974 (l), only 8 cases have, to our
knowledge, been described in Europe (2-5) and 4 In
the United States (6,7). In contrast, an entire session
of the 25th Meeting of the Japanese Society of Rheumatology (Kobe, May 21-22, 1981 [8]), which 1 of the
authors attended, was devoted to the study of SCCH
and its association with palmoplantar pustulosis. This
Figure 4. Lateral radiographs of the cervical spine. a, 1969: practically normal findings. (Note
that the lower part of the cervical spine was already masked by the fixed elevated posture of the
shoulder girdle.) b, 1982: new bone formation on the anterior aspect of the middle and lower
portions of the cervical spine.
session comprised 14 papers describing almost 200
cases, not including the recent publications of Sonozaki et a1 (9,lO). This illustrates well the particular
frequency of this condition in Japan.
Figure 5. Standard radiograph of the sacroiliac joints, 1982 (anteroposterior view after Barsony). The integrity of both sacroiliacs is
Our case is similar to most of those described in
the literature, except that in our patient there is no
doubt about the existence of psoriasis. The histology
of the elbow lesions was typical of this dermatosis, and
that of the plantar lesions presented the characteristics
of a spongiform pustulosis. These elements allow one
to make the certain diagnosis of pustular psoriasis of
Barber type ( 1 1 ) . It appears that in Japan, psoriasis
vulgaris is much rarer than it is in the western patients,
and the association between psoriasis vulgaris (regular
plaque psoriasis) and palmoplantar pustulosis is still
more exceptional (12). On the basis of HLA antigen
typing, the latter should be considered an entity distinct from psoriasis (13,14).
It is known that in axial forms of arthritis
associated with Reiter’s syndrome, and especially
with psoriasis, the inflammatory osteoarticular lesions
have a remarkable tendency to resolve by fibrosis,
with rapid ossification and bony ankylosis of diarthroses and synchondroses (15-18). Another characteristic
of these rheumatic syndromes is their propensity to
induce, in the insertion areas, a paraarticular new bone
formation leading to the frequently observed “fluffy”
Figure 6. Pustular plantar lesions. A , The histologic examination shows an intraepidermal pustule formed by the confluence of multiple small
zones of spongiosis with neutrophil polyrnorphonuclear leukocytes. B. Low magnification. C, High magnification.
periostitis (15-18). The same peculiarities have been
observed at the sternocostoclavicular junction in
SCCH. For this reason, it is important to determine
whether the palmoplantar pustulosis which is associated with SCCH is a particular form of psoriasis, as in
the case of our patient. What remains difficult to
Figure 7. Histologic picture of the thickened anterior aspect of the right clavicle (hematoxylin-eosin staining. original
magnification x 120): a. ordinary light. b, polarized light. (The picture has been slightly raised to better show the bone
cortex.) Lower part: lamellar cortical bone. Upper part: remodeled bony mass delimited on the top by a free border and
essentially made of woven bone. Perforating fibers inserted into the cortex are also embedded into the newly formed bony
understand, even if all the cases of palmoplantar
pustulosis were psoriatic dermatoses, is the very peculiar tropism of the osteoarticular lesions for the sternocostoclavicular junction.
If it seems possible to draw radiologic and
histologic parallels between the osteoarticular lesions
of SCCH and those of ankylosing spondylitis, the axial
forms of Reiter’s syndrome, and psoriasis, one should
expect to find an increased frequency of the HLA-B27
antigen in SCCH, since it is observed in these seronegative spondylarthropathies (16,19,20). Of the 13 cases
published and coming from western countries (including our patient), only 3 have been typed for the
histocompatibility antigens and of these 3, 2 have
HLA-B27. Therefore a correlation between this antigen and SCCH, in patients in the west, cannot be
Zachariae et a1 (21) have shown that the HLAB27 antigen is significantly more frequent when the
arthropathy is associated with a pustular form of
psoriasis (W,versus 21.6% in the vulgaris form).
Careful analysis of the results given in their report
reveals that pustular psoriasis is more often accompanied by an axial form of arthropathy (7 of 10 cases)
than is psoriasis vulgaris (13 of 34 cases).
Conversely in Japan, Kawashima in 94 cases
(14) and Sonozaki et al in 16 and later 36 cases (9,lO)
have not found any relationship between SCCH (either
isolated or existing in the context of a pustulotic
arthroosteitis) and the HLA-B27 antigen. This difference is all the more interesting to emphasize since in
Japan this antigen, although having a frequency of less
than 1% in the normal population, is present in 74% of
patients with ankylosing spondylitis, as it is in Reiter’s
syndrome and the axial arthropathy associated with
psoriasis (22).
Zachariae and coworkers have found that pustular psoriasis, whether accompanied or not by arthritis, differs from psoriasis vulgaris in that it has no
relation to HLA-B13 and B17 (21). In Japan, Sonozaki
et al (9) also have not observed an increase in the
frequency of these 2 antigens in their 53 cases of
pustulotic arthroosteitis.
The cause and pathogenesis of this peculiar
SCCH syndrome remain unknown for the moment. Its
clinical picture and radiologic manifestations are so
characteristic that it seems justified to deem it a
distinct entity. Sonozaki et al (9) consider it close t o
the group of seronegative spondylarthropathies, but
the relationship between them remains to be clarified.
Acknowledgments. We wish to thank Dr. J. Garcia
for his assistance with interpretation of the radiographs. We
are indebted to Dr. J.-M. Meyer, who performed the bone
biopsies and to Professors R. Lagier and A. Baud, who
carried out the histopathologic and microradiographic examinations. Professor J.H. Saurat and Dr. P. Chavaz were
kind enough to perform the dermatologic examination of the
patient and to interpret with their competent criticism the
skin biopsies. We are grateful to Dr. M. Jeannet and his
technicians, who conducted the HLA antigen typing. Dr. S.
Izui assisted us in translating from Japanese the abstracts on
SCCH presented at the 25th Meeting of the Japanese Society
of Rheumatology, and Mrs. D. Baer assisted in revising the
English text.
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