sternocostoclavicular hyperostosis in sapho-syndrome - rbrs

JBR–BTR, 2005, 88: 158-159.
STERNOCOSTOCLAVICULAR HYPEROSTOSIS IN SAPHO-SYNDROME
M. Vermaat, A.M. De Schepper, J.L. Bloem 1
Key-word: Bones, infection
Background: A 30-year-old woman was referred to the department with a history of progressive
pain and redness of the manubrial region of six months duration with restricted movements of
the shoulder girdle. Especially arm raising was a major problem. She also suffered of an extensive
acne and palmoplantar pustulosis.
A
A
B
1. Department of Radiology, Leids Universitair Medisch Centrum, The Netherlands
B
1A 1B
Fig. 2A
2B
3

Work-up
Coronal CT scan through the sterno-costo-clavicular
region (Fig. 1 A,B) shows sclerosis in the
manubrium (the right side being more affected)
and manubriocostal ossification.
Transverse CT scan through the sterno-costoclavicular
region (Fig. 2 A,B) shows pronounced
sclerosis (increased attenuation) in the manubrium
and manubriocostal synchondrosis with foci of
ossification. Both clavicles have a normal radiological
aspect.
Bone scintigraphy (Fig. 3) demonstrates a typical
“bull head “ pattern of intense radionuclide
uptake in the sterno-costo-clavicular region.
Radiological diagnosis
The radiological findings in association with the
dermatological abnormalities are consistent with
the diagnosis sternocostoclavicular hyperostosis
as part of the SAPHO - syndrome.
Discussion
STERNOCOSTOCLAVICULAR HYPEROSTOSIS IN SAPHO-SYNDROME — VERMAAT et al. 159
The Sapho syndrome includes osteoarticular
manifestations of synovitis, osteitis and hyperostosis
which may be associated with skin lesions,
mainly acne and palmoplantar pustulosis. SAPHO
stands for synovitis, acne, pustulosis, hyperostosis
and osteitis. The etiology and pathogenesis are
unclear. A faulty or atypical immune response to
viral or bacterial antigens is mentioned. The major
site of involvement is the anterior chest wall with
less common involvement of the spine, long
bones, small bones and large and small joints. All
the components of the anterior chestwall can be
involved, particularly the sternoclavicular, the
upper costosternal, the costochondral and the
manubriosternal junctions. The earliest clinical
signs of sternocostoclavicular hyperostosis (SCCH)
are swelling and redness, mainly localised to the
manubrial region. Pain, swelling and redness are
due to a destructive inflammatory process which
may spread to the clavicles and the anterior rib
segments. It involves the cortex and the medullary
canal with associated endosteal and periosteal
thickening. Reactive reparation and ankylosing
ossification lead to the radiological findings. Joint
erosions are frequently seen as a result of the primary
arthritis or an extension of the osteitis.
Histological examination shows a non-specific
chronic inflammatory reaction. Nuclear medicine
is a highly specific tool in the diagnosis of SCCH.
The characteristic “bull head” pattern of intense
radionuclide uptake is a specific sign and confirms
the diagnosis. The radiologist plays a key role in
the diagnosis. Osteomyelitis, osteosarcoma and
Paget’s disease can have similar radiological features
but dermatological abnormalities with typical
bone lesions in characteristic target sites or
bone scintigraphy with the presence of a bull’s
head sign must set you on thinking.
Bibliography
1. Earwaker J.W.S., Cotten A.: SAPHO: syndrome
or concept? Imaging findings. Skel Radiol, 2003,
311-327.
2. Freyschmidt J., Freischmidt G.: Pustulotic Arthroosteitis
(PAO) (Chapter 3). In: Freischmidt J., ed.
skibo- Diseases- Disorders affecting the Skin and
Bones, Verlag Berlin Heidelberg: Springer, 1999,
101-110.