Diffuse Chronic Sclerosing Osteomyelitis of the Mandible With

1

1

1

1

1

2

2

2

2

J6

Mimcdeasaom

R

U

e

S

D

M

s

g

©

0

d

212 MANDIBULAR OSTEOMYELITIS IN SAPHO SYNDROME

5. Raibley SO, Beckett RP, Nowakowski A: Multiple traumaticbone cysts of the mandible. J Oral Surg 37:335, 1979

6. Magliocca KR, Edwards SP, Helman JI: Traumatic bone cyst ofthe condylar region: Report of 2 cases. J Oral Maxillofac Surg65:1247, 2007

7. Wright JG, Yandow S, Donaldson S, et al: A randomized clinicaltrial comparing intralesional bone marrow and steroid injec-tions for simple bone cysts. J Bone Joint Surg Am 90:722, 2008

8. Capanna R, Dal Monte A, Gitelis S, et al: The natural history ofunicameral bone cyst after steroid injection. Clin Orthop RelatRes 166:204, 1982

9. Scaglietti O, Marchetti PG, Bartolozzi P: Final results obtained in
the treatment of bone cysts with methylprednisolone acetate
Izumi Yoshitomi, DDS, PhD,‡ an

cBcoi1tastittoms

cw(i

R

Oavoi:10.1016/j.joms.2009.04.121

(depo-medrol) and a discussion of results achieved in other bonelesions. Clin Orthop Relat Res 165:33, 1982

0. Hansen LS, Scapone J, Sprout C: Traumatic bone cysts of jaws:Report of sixty-six cases. Oral Surg Oral Med Oral Pathol 37:899, 1974

1. Wilkins R: Unicameral bone cysts. J Am Acad Orthop Surg8:217, 2000

2. Oppenheim WL, Galleno H: Operative treatment versus steroidinjection in the management of unicameral bone cysts. J PedOrthop 4:1, 1984

3. Yu CL, D’Astous J, Finnegan M: Simple bone cysts. The effectsof methylprednisolone on synovial cells in culture. Clin Orthop
Relat Res 34, 1991
Oral Maxillofac Surg8:212-217, 2010

Diffuse Chronic Sclerosing Osteomyelitisof the Mandible With Synovitis, Acne,Pustulosis, Hyperostosis, and Osteitis:Report of a Long-Term Follow-Up Case

Souichi Yanamoto, DDS, PhD,* Goro Kawasaki, DDS, PhD,†

d Akio Mizuno, DDS, PhD§

andibular osteomyelitis is one of the most commonnfectious diseases and is usually odontogenic or trau-

atic in origin. Meanwhile, mandibular osteomyelitisaused by a process of unknown etiology is known toevelop during the clinical course. In 1987, Chamott al1 described a syndrome associated with synovitis,cne, pustulosis, hyperostosis, and osteitis (SAPHOyndrome), which is characterized by osteoarticularnd dermatologic symptoms.2 The most prevalent sitef bone lesions is the anterior chest wall with involve-ent of other locations including the sternum, clavi-

eceived from the Department of Oral and Maxillofacial Surgery,

nit of Translational Medicine, Course of Medical and Dental Sci-

nces, Nagasaki University Graduate School of Biomedical Sciences,

akamoto, Nagasaki, Japan.

*Senior Assistant Professor.

†Associate Professor.

‡Assistant Professor.

§Professor and Chairman.

Address correspondence and reprint requests to Dr Yanamoto:

epartment of Oral and Maxillofacial Surgery, Unit of Translational

edicine, Course of Medical and Dental Sciences, Nagasaki Univer-

ity Graduate School of Biomedical Sciences, 1-7-1 Sakamoto, Na-

asaki, 852-8588, Japan; e-mail: [email protected]

2010 American Association of Oral and Maxillofacial Surgeons

278-2391/10/6801-0036$36.00/0

les, ribs, spine, and peripheral long and flat bones.1-4

one lesions in SAPHO syndrome demonstrate clini-al and radiologic features similar to diffuse sclerosingsteomyelitis.5 Clinical diagnosis of SAPHO syndrome

s defined as the presence of any one of the following:) multifocal osteitis with or without skin manifesta-ions; 2) sterile acute or chronic joint inflammationssociated with pustules or psoriasis of palms andoles, or acne, or hidradenitis; or 3) sterile osteitis inhe presence of one of the skin manifestations.6 Othernvestigators have suggested that early diagnosis ofhis condition is crucial to avoid repeated examina-ions and invasive procedures; however, the etiologyf SAPHO syndrome remains unknown.1,2,5-7 Treat-ent has therefore been difficult and focuses on

ymptoms only.3,5,7

This report presents the long-term follow-up of aase of SAPHO syndrome in the mandible of a patientho received nonsteroid anti-inflammatory drugs

NSAIDs) and long-term administration of macrolidesn combination with surgical procedures.

eport of a Case

A 51-year-old woman was referred to the Department ofral and Maxillofacial Surgery, Nagasaki University Gradu-

te School of Biomedical Sciences (Nagasaki, Japan) in No-
ember 1998 because of a painful swelling of the right
mailto:[email protected]

cwpm

mc

rmlscel

datscntsbtac

srpotetCn

Ft

YJ

Fp

Y

YANAMOTO ET AL 213

heek associated with limited mouth opening for at least 3eeks. She did not have weakness or fever. At that time, theatient reported no use of medications or previous treat-ent for these conditions. Her medical history was unre-

IGURE 1. Extraoral photograph shows a bony hard swelling inhe region of the right parotid masseter.

anamoto et al. Mandibular Osteomyelitis in SAPHO Syndrome.Oral Maxillofac Surg 2010.

IGURE 2. Panoramic radiogram (A) and coronal TMJ tomogramrocess of the mandible.

anamoto et al. Mandibular Osteomyelitis in SAPHO Syndrome. J Oral

arkable. There was no history of trauma to the maxillofa-ial complex.Clinical examination showed a bony hard swelling in the

egion of the right parotid-masseter and the right temporo-andibular joint (TMJ) without suppuration and cervical

ymphadenopathy (Fig 1). Intraorally, no alterations wereeen in the oral mucosa. Fever did not increase during theourse. In laboratory data, C-reactive protein was slightlylevated (2.3 mg/dL); however, blood counts and otheraboratory tests were within normal limits.

Panoramic radiogram and coronal TMJ tomogram showedestruction of the condyle and reactive sclerosis of therticular process of the mandible (Figs 2A,B). Coronal con-rast-enhanced T1-weighted magnetic resonance imagehowed a low intensity signal of the bone marrow of theondyle and ascending ramus (Figs 3A,B). Based on a diag-osis of mandibular osteomyelitis, antibiotic and NSAIDherapy was given for 1 week, but it failed to improve theymptoms. Partial resection of the condyle with an openiopsy was performed under general anesthesia. The his-opathologic examination showed fibrous granulation tissuend mature lamellate cellular bone (Fig 4). Microbiologiculture from the biopsy specimen was negative.Two years later, the patient also experienced pain and

welling in the right mandibular body, but no evidence ofecurrence of the mouth-opening limitation. At that time,anorama radiogram showed bone sclerosis with scatteredsteolyses of the ascending ramus (Fig 5A). Coronal TMJomogram showed cortex formation of the condyle; how-ver, the progressive sclerotic change and periosteal reac-ion were predominant in the ascending ramus (Fig 5B).oronal contrast-enhanced T1-weighted magnetic reso-ance image demonstrated extension of the low intensity

w destruction of the condyle and reactive sclerosis of the articular
(B) sho
Maxillofac Surg 2010.

sp(hvidnflaCid

dstbq(Dts

iasdecpotscm

D

imftidocsmao

sjeS(wpl

Ft

Fot

YJ


ignal of the bone marrow to the mandibular angle, and theeriosteal reaction was observed outside the original cortexFig 6). Bone scintigram (technetium 99m) showed en-anced uptake in the right ascending ramus and sternocla-icular joint (Fig 7). In laboratory data, as in the first exam-nations, C-reactive protein was slightly increased (0.55 mg/L); however, other laboratory test results were withinormal limits. In addition, she was negative for rheumatoidactor and antinuclear antibody. Serum immunoglobulinevels and immunoglobulin G subclasses were normal. HLAntigen typing was positive for A11, A24, B55, B52, andw1. Based on the clinical manifestations, laboratory exam-

nations, and radiologic findings, a diagnosis of SAPHO syn-rome was established.Extensive decortication of the ramus was performed to

ecrease swelling of the mandible. Histopathologic findingshowed fibrous granulation tissue and bone fragments, as inhe first surgical treatment. Microbiologic culture of theiopsy specimen was also negative. The patient subse-uently received long-term administration of clarithromycin400 mg/d) and etodolac (200 mg/d) for at least 3 months.uring that time, she had a long symptom-free period. At

his point, the antibiotic and NSAID treatments were

IGURE 3. Coronal contrast-enhanced T1-weighted magnetic res-nance image shows a low intensity signal of the bone marrow of

he condyle (A) and ascending ramus (B).


topped.YJ

After 6 months, she again experienced pain and swellingn the right mandibular body; however, the degree of painnd swelling were markedly decreased. Subsequently, theseymptoms appeared every 3 months and the patient wasiscontinuously administered clarithromycin (400 mg/d) andtodolac (200 mg/d). This symptomatic treatment has beenontinuing for the past 8 years, resulting in sufficient relief ofain and swelling. At the last radiologic examination, pan-ramic radiogram showed slight enhancement of sclerosis ofhe ascending ramus (Fig 8A). Coronal TMJ tomogram showedlight enlargement of the ascending ramus (Fig 8B). Coronalontrast-enhanced T1-weighted magnetic resonance image re-ained almost unaltered (Fig 9).

iscussion

SAPHO syndrome is a rare disease of unknownnfectious origin; however, its incidence is underesti-

ated. A pediatric subset of SAPHO syndrome is re-erred to as chronic recurrent multifocal osteomyeli-is,8 which is the most severe form of sterile bonenflammation in children.9 Moreover, SAPHO syn-rome may be misdiagnosed if the location is atypicalr if the clinical presentation is unusual. In fact,hronic recurrent multifocal osteomyelitis and SAPHOyndrome share several features: osteitis, unifocal orultifocal presentation, pustulosis, hyperostosis, andgood general state of health without spiking fevers,rganomegaly, weight loss, or fatigue.8

A sternocostoclavicular lesion is the most frequentite of SAPHO syndrome, followed by the sacroiliacoint and the spine.10,11 Diffuse sclerosing osteomy-litis of the mandible is a well-known bone lesion ofAPHO syndrome. Hayem et al11 reported 13 cases10.8%) of mandibular osteomyelitis in 120 patientsith SAPHO syndrome. Moreover, in a series of 85atients with SAPHO syndrome, 7 had mandibular

esions (8.2%).6 Despite previous reports of SAPHO

IGURE 4. Histopathologic examination shows fibrous granula-ion tissue and mature lamellate cellular bone.


siplctr

do

em

Fu

Frp

Y J Oral

Fobi

YJ

YANAMOTO ET AL 215

yndrome in the literature,6,7,11-13 only 4 cases involv-ng the TMJ have been reported.7,11-13 These casesresented various complications such as TMJ anky-

osis7 and inflammatory spread to the temporal boneausing deafness.12 Although our case also involvedhe TMJ and showed destruction of the condyle andeactive sclerosis of the articular process of the man-

IGURE 5. Two years after first surgical treatment. A, Panoramic raamus. B, Coronal TMJ tomogram shows cortex formation of the coredominant in the ascending ramus.

anamoto et al. Mandibular Osteomyelitis in SAPHO Syndrome.

IGURE 6. Coronal contrast-enhanced T1-weighted magnetic res-nance image shows extension of the low intensity signal of theone marrow to the mandibular angle, and the periosteal reaction

s observed outside the original cortex.

YJ


ible, good progress was achieved by partial resectionf the condyle as the initial surgical procedure.Suei et al5 recommended that mandibular osteomy-

litis lesions should be classified into bacterial osteo-yelitis and osteomyelitis in SAPHO syndrome. Diagnos-

IGURE 7. Technetium 99m bone scintigram shows enhancedptake in the right ascending ramus and sternoclavicular joint.

m shows bone sclerosis with scattered osteolyses of the ascendinghowever, progressive sclerotic change and periosteal reaction is


diograndyle;


tape

pnrSsttoassc

ptoartoh

bkad

Fe

Y J Oral

Fcms

YJ


ic features of bacterial osteomyelitis are suppurationnd osteolytic radiographic change with lamellar-typeeriosteal reaction. In contrast, mandibular osteomy-litis in SAPHO syndrome is characterized by nonsup-

IGURE 8. Eight years after second surgical treatment. A, Atnhancement of sclerosis of the ascending ramus. B, Coronal TMJ

anamoto et al. Mandibular Osteomyelitis in SAPHO Syndrome.

IGURE 9. Eight years after second surgical treatment. Coronalontrast-enhanced T1-weighted magnetic resonance image re-ained almost unaltered from that seen in Figure 6 (2 yrs after first

urgical treatment).

ianamoto et al. Mandibular Osteomyelitis in SAPHO Syndrome.Oral Maxillofac Surg 2010.

uration and a mixed radiographic pattern accompa-ied by solid-type periosteal reaction, external boneesorption, and bone enlargement.5,10,14 Moreover, inAPHO syndrome, progressive bone sclerosis withcattered osteolyses (mixed type) is a common fea-ure5; however, bone resorption may be prominent inhe early stage, whereas sclerotic changes may bebserved in a more quiescent chronic reaction.5 Inccordance with these radiologic features, our casehowed bone resorption of the condyle in the earlytage, and sclerotic changes were observed withhronic inflammation in recent years.Skin lesions typically seen in SAPHO syndrome are

almoplantar pustulosis and acne; however, not all ofhese manifestations necessarily occur. The incidencef skin lesions such as palmoplantar pustulosis andcne in patients with SAPHO syndrome has beeneported as 84%.11 Moreover, Kahn et al15 reportedhe occurrence of long intervals between the devel-pment of skin and bone lesions; however, our casead no history of skin lesions.SAPHO syndrome shows various immunogenetic

ackgrounds; however, its genetic basis remains un-nown.16 Although some studies have suggested thatntigen HLA B27 is associated with SAPHO syn-rome,1,6 some recent reports have supported the

t radiologic examination, panoramic radiogram showed slightram showed slight enlargement of the ascending ramus.


the lastomog

dea that the HLA B27 phenotype could not be associ-

aO

artshetisdUctbaNtNtbonItms

smtact

A

Bl

R

1

1

1

1

1

1

1

1

1

YANAMOTO ET AL 217

ted with the pathogenesis of SAPHO syndrome.11,16

ur patient did not have the HLA B27 phenotype.In SAPHO syndrome, therapeutic modalities such

s antibiotic administration, hyperbaric oxygen, cu-ettage, saucerization, decortication, and partial resec-ion of the affected bones have been reported byome investigators3,7,11,13; however, these modalitiesave very limited efficacy and cannot cure the dis-ase.5,13 Although surgical procedures such as decor-ication and removal of necrotic tissue may be usefuln the early stages of the disease, it is well known thaturgical treatment of osteomyelitis with SAPHO syn-rome often has no or only short-term success.17,18

p to now, treatment for SAPHO syndrome has fo-used only on symptoms. NSAIDs seem to be thereatment of first choice, in combination with anti-iotics.3,7,11,13,18 Corticosteroid therapy is accept-ble for patients with a poor clinical response toSAIDs.11,13 In almost all cases, long-term administra-

ion of macrolides and conservative treatment withSAIDs have been recommended.5,7,11,18 Recently,

reatment with pamidronates or bisphosphonates haseen reported to be effective11; however, the effectsf these drugs have to be evaluated, because there areo long-term data on the outcome with these drugs.n our case, NSAIDs (etodolac 200 mg/d) and long-erm macrolides (clarithromycin 400 mg/d) were ad-inistered. Moreover, surgical treatment in the early

tage seemed effective to repress disease progression.In conclusion, mandibular osteomyelitis in SAPHO

yndrome is characterized by nonsuppuration and aixed radiographic pattern accompanied by solid-

ype periosteal reaction, external bone resorption,nd bone enlargement. SAPHO syndrome should beonsidered when a patient presents with osteomyeli-is in other bones, arthritis, or skin diseases.

cknowledgment

We thank the staff of the Department of Radiology and Canceriology and the Department of Oral Pathology and Bone Metabo-

ism, Nagasaki University Graduate School of Biomedical Sciences.

eferences1. Chamot AM, Benhamou CL, Kahn MF, et al: Acne-pustulosis-

hyperostosis-osteitis syndrome. Results of a national survey. 85
Cases. Rev Rhum Mal Osteoa-Articulaires 54:187, 1987
2. Earwaker JWS, Cotton A: SAPHO: Syndrome or concept? Imag-ing findings. Skeletal Radiol 32:311, 2003

3. Roldan JC, Terheyden H, Dunsche A, et al: Acne with chronicrecurrent multifocal osteomyelitis involving the mandible aspart of the SAPHO syndrome: Case report. Br J Oral MaxillofacSurg 39:141, 2001

4. Sato T, Indo H, Kawabata Y, et al: Scintigraphic evaluation ofchronic osteomyelitis of the mandible in SAPHO syndrome.Dentomaxillofac Radiol 30:293, 2001

5. Suei Y, Taguchi A, Tanimoto K: Diagnosis and classification ofmandibular osteomyelitis. Oral Surg Oral Med Oral Pathol OralRadiol Endod 100:207, 2005

6. Kahn MF, Hayem F, Hayem G, et al: Is diffuse sclerosingosteomyelitis of the mandible part of the synovitis, acne, pus-tulosis, hyperostosis, osteitis (SAPHO) syndrome? Analysis ofseven cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod78:594, 1994

7. Utumi ER, Sales MAO, Shinohara EH, et al: SAPHO syndromewith temporomandibular joint ankylosis: Clinical, radiological,and therapeutical correlations. Oral Surg Oral Med Oral PatholOral Radiol Endod 105:e67, 2008

8. Jansson A, Renner ED, Ramser J, et al: Classification of non-bacterial osteitis. Rheumatology 46:154, 2007

9. Girschick HJ, Raab P, Surbaum S, et al: Chronic non-bacterialosteomyelitis in children. Ann Rheum Dis 64:279, 2005

0. Maugars Y, Berthelot JM, Ducloux JM, et al: SAPHO syndrome:A follow up study of 19 cases with special emphasis on enthe-sis involvement. J Rheumatol 22:2135, 1995

1. Hayem G, Bouchaud-Chabot A, Benali K, et al: SAPHO syn-drome: A long-term follow-up study of 120 cases. Semin Arthri-tis Rheum 29:159, 1999

2. Marsot-Dupuch K, Doyen JE, Grauer WO, et al: SAPHO syn-drome of the temporomandibular joint associated with suddendeafness. AJNR 20:902, 1999

3. Eyrich GK, Langenegger T, Bruder E, et al: Diffuse chronicsclerosing osteomyelitis and the synovitis, acne, pustulosis,hyperostosis, osteitis (SAPHO) syndrome in two sisters. IntJ Oral Maxillofac Surg 29:49, 2000

4. Suei Y, Taguchi A, Tanimoto K: Diagnostic points and possibleorigin of osteomyelitis in synovitis, acne, pustulosis, hyperos-tosis and osteitis (SAPHO) syndrome: A radiographic study of77 mandibular osteomyelitis cases. Rheumatology 42:1398,2003

5. Kahn MF, Bouvier M, Palazzo E, et al: Sternoclavicular pustu-lotic osteitis (SAPHO). 20-Year interval between skin and bonelesions. J Rheumatol 18:1104, 1991

6. Queiro R, Moreno P, Sarasqueta C, et al: Synovitis-acne-pustu-losis-hyperostosis-osteitis syndrome and psoriatic arthritis ex-hibit a different immunogenetic profile. Clin Exp Rheumatol26:125, 2008

7. Suei Y, Tanimoto K, Miyauchi M, Ishikawa T: Partial resectionof the mandible for the treatment of diffuse sclerosing osteo-myelitis: Report of four cases. J Oral Maxillofac Surg 55:410,1997

8. Baltensperger M, Gratz K, Bruder E, et al: Is primary chronicosteomyelitis a uniform disease? Proposal of a classificationbased on a retrospective analysis of patients treated in the past
30 years. J Craniomaxillofac Surg 32:43, 2004

Diffuse Chronic Sclerosing Osteomyelitis of the Mandible With

Documents

Transcript of Diffuse Chronic Sclerosing Osteomyelitis of the Mandible With