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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 9  |  Issue : 1  |  Page : 5-9

SAPHO syndrome of the right temporomandibular bone and joint in a 25-year-old man


1 Department of Radiology, Faculty of Clinical Science, College of Medical Science, Ahmadu Bello University, Zaria, Nigeria
2 Department of Medicine, Faculty of Clinical Science, College of Medical Science, Ahmadu Bello University, Zaria, Nigeria

Date of Submission07-Nov-2019
Date of Acceptance19-Feb-2020
Date of Web Publication16-Apr-2020

Correspondence Address:
Dr. Bello O Usman
Department of Radiology, College of Medical Sciences, Ahmadu Bello University, Zaria
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ais.ais_34_19

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  Abstract 


SAPHO syndrome is an association of specific bone, joint, and skin lesions. The acronym SAPHO refers to synovitis, acne, palmoplantar pustulosis, hyperostosis, and osteitis. However, not all these clinical manifestations need to be present to establish the diagnosis. It is a rare disease in which genetic predisposition or an autoimmune response to a microorganism in the skin mimicking a normal bone or joint molecular structure has been proposed. We present a 25-year-old man who presented with 2-year history of difficulty in opening the mouth with associated pain in the right jaw. He also developed progressive right hearing impairment and multiple acne-like rashes on his face 10 months prior to presentation. Examination of the right jaw revealed a tender firm immobile swelling in the region of the temporomandibular joint which was slightly warm to touch. CECT revealed cortical bone erosions of the right temporal squama and condyle with diffuse endosteal sclerosis and hyperostosis of the mandibular condyle and ramus. The temporal squama was partially resorbed with areas of bone remodeling. The right temporomandibular joint was also involved in the sclerotic changes with marked narrowing of its joint space. Based on the clinical and radiological features, a diagnosis of SAPHO syndrome was established. It is essential to make such diagnosis in order to avoid unnecessary and futile treatment.

Keywords: Jaw swelling, SAPHO syndrome, temporomandibular joint synovitis


How to cite this article:
Usman BO, Oyewole FA, Umar A. SAPHO syndrome of the right temporomandibular bone and joint in a 25-year-old man. Arch Int Surg 2019;9:5-9

How to cite this URL:
Usman BO, Oyewole FA, Umar A. SAPHO syndrome of the right temporomandibular bone and joint in a 25-year-old man. Arch Int Surg [serial online] 2019 [cited 2020 Jul 12];9:5-9. Available from: http://www.archintsurg.org/text.asp?2019/9/1/5/282576




  Introduction Top


The term SAPHO syndrome was accepted in 1987[1] to describe an association of characteristic bone, joint, and skin lesions. The acronym SAPHO refers to synovitis, acne, palmoplantar pustulosis, hyperostosis, and osteitis. Not all these clinical manifestations need to be present to establish the diagnosis.[1]

The association of osteitis and cutaneous lesions is sufficient for the diagnosis of SAPHO syndrome, its prevalence is not more than 1/10,000 and can be seen in all ages, mandibular manifestation has been described in 10% of cases, but skin lesions may be absent or appear after some delay or may be so subtle as to escape attention.[2],[3]

The bone lesions are similar to those reported by stomatologists for years as diffuse sclerosing osteomyelitis of the mandible (DSOM).[4]

SAPHO syndrome is a rare disease of unknown origin.[5] The theory of a genetic predisposition or an autoimmune response to a microorganism in the skin mimicking a normal bone or joint molecular structure has been proposed.[2]

Natural history of disease includes multiple osteoarticular involvement and periodic exacerbations and remissions. Despite the above theory, no pathogen or microorganism has been isolated in the affected regions of synovial and osseous inflammation.[3],[6]

Radiologists play some vital role in the diagnosis of SAPHO syndrome, though it be asymptomatic. Radiologic findings in SAPHO syndrome include: osteolysis, osteitis, hyperostosis, and osteosclerosis. Joint erosions involving adjacent articulations are frequently seen as a result of primary arthritis or extension of adjacent osteitis. Osteitis usually presents as osteosclerosis with homogenous fibrillary pattern.[7]

Hyperostosis, which is highly characteristic of SAPHO, is characterized by chronic periosteal reaction and cortical thickening, resulting in bone hypertrophy. CT and MRI are helpful in locating the lesions and providing information on adjacent soft tissues.

Report of this rare case is to increase awareness about SAPHO syndrome among patients, followed by early accurate diagnosis which obviates misdiagnosis, unnecessary biopsy, or antibiotic therapy.


  Case Report Top


A 25-year-old man presented at the General Outpatient Clinic of the Ahmadu Bello University Teaching Hospital (ABUTH), Zaria on account of 2-year history of difficulty in opening the mouth with associated pain in the right jaw. He also developed progressive right hearing impairment and multiple acne-like rashes on his face 10 months prior to presentation. The pain was recurrent and sharp in nature, with radiation to the cheek. No definite aggravating or relieving factors were noted. However, the symptoms were associated with occasional fever but no history of significant weight loss. There was no previous history of loss of consciousness, irradiation, or head trauma. There was no symptom referable to the cardio-pulmonary or gastrointestinal systems. The patient is the third of nine children from polygamous parents. There was no history of similar illness in any member of the family. There was past medical history of several episodes of right jaw pain treated at peripheral hospitals with courses of antibiotics without any appreciable improvement.

On general physical examination, he was afebrile, not pale, anicteric, not dehydrated, not irritable or in respiratory distress. No peripheral edema but had inguinal lymphadenopathy. Examination of the right jaw revealed a tender firm immobile swelling in the region of the temporomandibular joint which was slightly warm to touch. The clinical impression was adamantinoma, with a differential diagnosis of diffuse sclerosing osteomyelitis.

Liver and renal function tests were normal. The erythrocyte sedimentation rate (ESR) and C- reactive protein were slightly raised. The precontrast CT slices were obtained. The images revealed cortical bone erosions of the right temporal squama and condyle with diffuse endosteal sclerosis and hyperostosis involving the mandibular condyle and ramus [Figure 1] and [Figure 2]. The temporal squama was partially resorbed with areas of bone remodeling. The right temporomandibular joint (TMJ) was also involved in the sclerotic changes with marked narrowing of its joint space [Figure 3]. No TMJ effusion was noticed. The ipsilateral inner and middle ear, and mastoid air cells also appeared sclerosed. The contralateral temporal bone and temporomandibular joint were preserved. The CT scanogram showed normal cranial vault, sutures, and sellar turcica [Figure 4]a. The cerebral and cerebellar hemispheres, ventricular system, and the basal cisterns were within normal limits [Figure 4]b.
Figure 1: Axial CT (bone windows) showing (a) osteosclerosis of the squamous, petrous, and mastoid parts of the right temporal bone with (b) marked reduction of the mastoid air cells (star) and hazy ipsilateral ear canal (arrow)

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Figure 2: Axial CT scan (a and b) showing osteosclerosis of the right temporal squama (arrow) and the petrous temporal bone housing the middle and inner ear. There is also marked reduction of the mastoid air cells when compared with the left side

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Figure 3: Sagittal (a) and coronal (b) reformatted computerized tomographic images showing hazy/sclerosis of the right temporomandibular joint with narrowed joint space (long arrow). Diffuse osteosclerosis of the right mandibular condyle is also noted (short arrow)

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Figure 4: CT scanogram (a) and noncontrast axial (b) CT scans showing normal cranial vault, sutures, and the sellar turcica. The brain mantle and the ventricular system are also preserved

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  Discussion Top


SAPHO syndrome is characterized by osteoarticular and dermatological manifestations. Hurtado-Nedelec et al.[1] described the syndrome in 2008 in the article “le syndrome acne pustulose hyperostose osteite” and introduced the acronym SAPHO, to designate the constellation of these five frequently combined disorders. The syndrome can occur at any age but usually appears between childhood and middle age, and follows a prolonged relapsing and remitting course.[7] However, our patient is in his mid-twenties.

The pathogenesis of SAPHO is not clear.[6] Infectious and post-infectious theories were considered and it was reported that propionibacterium acnes, could be related to SAPHO Syndrome, but investigations of most of the cases regarding Propionibacterium Acne were negative and generally, patients do not respond to antibiotic therapy,[7] as seen in the current patient.

Diagnosis of SAPHO Syndrome is more often based on clinical findings. There is a laid down diagnostic criteria for SAPHO Syndrome. Diagnostic criteria for SAPHO syndrome were suggested for the first time by Hurtado-Nedelec et al.[1] These criteria were later updated in 2003 at the 67th annual scientific meeting of American College of Rheumatology. The inclusion criteria are: a) bone and/or joint involvement associated with palmoplantar pustulosis and pustular psoriasis, b) bone and/or joint involvement associated with severe acne, c) in adults isolated sterile hyperostosis/osteitis, d) in children chronic recurrent multifocal osteomyelitis, and e) bone and/or joint involvement associated with chronic bowel diseases. From the above criteria, the present patient belongs to the second group due to the presence of hyperostosis of the right mandibular condyle and the temporal bone with associated TMJ involvement and facial acnes. Exclusion criteria are: a) infectious osteitis, b) tumoral conditions of bone, and c) noninflammatory condensing lesions of bone.[8] Thus, the diagnosis of SAPHO syndrome is based on the exclusion of infectious arthritis or osteomyelitis, and the presence of at least one of four diagnostic criteria proposed by Laiho et al.[9]

The prevalence of SAPHO syndrome is not known completely because it is a new concept and has mixed clinical findings. It is estimated that its prevalence is not more than 1/10,000. Published reports of SAPHO syndrome are generally from France, Germany, Japan, Australia, and Scandinavian countries.[3],[6] There was a reported case in African American.[10]

Hyperostosis and osteitis are the most prominent SAPHO syndrome findings,[2] and these were well demonstrated in this patient [see [Figure 1] and [Figure 2]. Hyperostosis appears radiologically as osteosclerosis, with cortical thickening, narrowing of the medullary canal, and external surface of the bone appearing expanded, indistinct, and irregular. Osteitis refers to bone inflammation that appears histopathologically as a sterile inflammatory infiltrate. Bacterial cultures are usually negative,[7] but low virulence microbial agents, such as  Propionibacterium acnes Scientific Name Search > have been isolated in deep bone biopsy specimens in a few cases, and Staphylococcus aureus (S. aureus) has been isolated from a plantar pustule in one case.[11]

Bone pain and dermatologic manifestations are the most troublesome SAPHO syndrome symptoms.[12] Bony lesions manifest as severe, recurrent, debilitating pain and tenderness. These symptoms can be more severe at night. Cutaneous manifestations include palmoplantar pustulosis (PPP), severe cystic acne, and pustular psoriasis. Skin lesions may precede, occur concurrently, or appear after onset of osteoarticular manifestations,[7] but may be initially absent in 1/3 of patients and consequently, absence of skin lesions does not exclude SAPHO syndrome.[13] The interval between onset of skin manifestations and osteoarticular lesions is generally less than 2 years, but intervals as long as 20 and 38 years have been reported.[7] Cutaneous manifestation in the present patient was mild with healing facial acnes seen.

Because of misdiagnosis or lack of diagnosis, patients with SAPHO syndrome often receive long-term antibiotic therapy combined with surgical procedures, such as saucerization, decortication, or partial resection of affected bones. Our patient also had past medical history of treatment with antibiotics at peripheral hospitals without any appreciable improvement and there was no surgical intervention.

Radiologic findings in SAPHO syndrome include osteolysis, osteitis, hyperostosis, and osteosclerosis.[1] Osteitis usually presents as osteosclerosis with a homogenous fibrillary pattern.[7] Hyperostosis, as seen in SAPHO, is characterized by chronic periosteal reaction and cortical thickening, resulting in bone hypertrophy. Joint erosions involving adjacent articulations are frequently seen as result of primary arthritis or extension of adjacent osteitis. Aforementioned features were well demonstrated on the CT scan images of our patient [see [Figure 4].

CT and MRI are helpful in locating the lesions and providing information on adjacent soft tissues, but cannot distinguish SAPHO lesions with osteomyelitis and malignancy. Bone scintigraphy is important in diagnosing SAPHO syndrome, particularly for detecting early bone involvement. MRI and bone scintigraphy were not done in this case due to nonavailability/financial constraints. Bone biopsy is often used in an attempt to establish a diagnosis. The bone biopsy done for our patient confirmed the diagnosis.

Bone lesion histopathology is variable, with early lesions characterized by the presence of polymorphonuclear infiltrate. Intermediate phase is characterized by chronic inflammation (primarily mononuclear cells), whereas lesions with markedly enlarged and sclerotic bone trabeculae and prominent marrow fibrosis are seen in late phases.[14]Propionibacterium acnes, a skin saprophyte, has been isolated from cutaneous lesions with severe acne and from articular and osseous lesions associated with pustulosis. However, most biopsies of involved areas demonstrate nonspecific inflammatory infiltrate,[15] and therefore cannot confirm the diagnosis.

Treatment options for SAPHO include: NSAIDs, antirheumatic drugs, such as colchicine, corticosteroids, and biphosphonates, and disease-modifying agents, such as methotrexate, sulfasalazine, and infliximab.[15] Antimicrobial therapies are a reasonable choice, since bacteria, such as P. acnes and S. aureus,[11] have been isolated in specimens from SAPHO patients and have been implicated in SAPHO pathogenesis.[11]

Azithromycin, doxycycline, sulfamethoxazole/trimetoprim, and clindamycin use have been reported in SAPHO.[11] Our patient was managed on outpatient basis with NSAIDs, corticosteroids, and methotrexate, with remarkable improvement in the clinical symptoms. No surgical intervention was done.

Surgery is only indicated when SAPHO-induced changes adversely affect bone mechanics.

The patient is still being followed up at the clinic with frequent clinic defaults.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Hurtado-Nedelec M, Chollet-Martin S, Nicaise-Roland P, Grootenboer-Mignot S, Ruimy R, Meyer O, et al. Characterization of the immune response in the synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome. Rheumatology 2008;47:1160-7.  Back to cited text no. 1
    
2.
Earwaker JW, Cotten AS. SAPHO: Syndrome or concept? Imaging findings. Skeletal Radiol 2003;32:311-27.  Back to cited text no. 2
    
3.
Van Doornum S, Barraclogh D, McColl G, Wicks I. SAPHO: Rare or just not recognized? Semin Arthritis Rheum 2000;30:70-7.  Back to cited text no. 3
    
4.
Suei Y, Taguchi A, Tanimoto K. Diagnosis and classification of mandibular osteomyelitis. Oral Surg Oral Med Oral Pathol Oral Radiol Endodontol 2005;100:207-14.  Back to cited text no. 4
    
5.
Laredo JD, Vuillemin-Bodaghi V, Boutry N, Cotten A, Parlier-Cuau C. SAPHO syndrome: MR appearance of vertebral involvement. Radiology 2007;242:825-31.  Back to cited text no. 5
    
6.
Hayem G. Valuable lessons from SAPHO syndrome. Joint Bone Spine 2007;74:123-6.  Back to cited text no. 6
    
7.
Amital H, Applbaum YH, Aamar S, Daniel N, Rubinow A. SAPHO syndrome treated with pamidronate: An open-label study of 10 patients. Rheumatology 2004;43:658-61.  Back to cited text no. 7
    
8.
Govoni M, Colina M, Massara A, Trotta F. SAPHO syndrome and infections. Autoimmun Rev 2009;8:256-9.  Back to cited text no. 8
    
9.
Laiho K, Soini I, Martio J. Magnetic resonance imaging findings of manubriosternal joint involvement in SAPHO syndrome. Clin Rheumatol 2001;20:232-3.  Back to cited text no. 9
    
10.
Merola JF, Callen J, Ofori AO. SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome. UpToDate. Retrieved on August. 2017;9: 2018.  Back to cited text no. 10
    
11.
Rozin AP, Nahir AM. Is SAPHO syndrome a target for antibiotic therapy? Clin Rheumatol 2007;26:817-20.  Back to cited text no. 11
    
12.
Marshall H, Bromilow J, Thomas AL, Arden NK. Pamidronate: A novel treatment for the SAPHO syndrome? Rheumatology (Oxford) 2002;41:231-3.  Back to cited text no. 12
    
13.
Nguyen MT, Borchers A, Selmi C, Naguwa SM, Cheema G, Gershwin ME. The SAPHO syndrome. In: Seminars in arthritis and rheumatism 2012 Dec 1 (Vol. 42, No. 3, pp. 254-265). WB Saunders.  Back to cited text no. 13
    
14.
Guignard S, Job-Deslandre C, Sayag-Boukris V, Kahan A. Pamidronate treatment in SAPHO syndrome. Joint Bone Spine 2002;69:392-6.  Back to cited text no. 14
    
15.
Olivieri I, Padula A, Ciancio G, Salvarani C, Niccoli L, Cantini F. Successful treatment of SAPHO syndrome with infliximab: Report of two cases. Ann Rheum Dis 2002;61:375-6.  Back to cited text no. 15
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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