HONG AND FARISH 435

used for implant removal. Moreover, removal of the implant would have subjected the patient to the morbidity of one surgical procedure to remove the implant and graft the area, followed by a repeated implant procedure in 3 months, and another waiting

sive, least morbid, and most cost- and time-effective way to solve the problem.

References period of 6 months for integration of the new implant. Thus, the procedure used represented the least inva-

1. Peterson LJ (ed): Principles of Oral and Maxillofacial Surgery. Philadelphia, PA, Lippincott, 1992, p 921

] Oral Maxillofac Surg

58 435.439,200O

Intraosseous Sarcoidosis of the MaxiUa: Case Report

Jay Hong, DDS, * and Sam E. Farish, DMDf

Sarcoidosis is a systemic, multiorgan, granulomatous disease in which discrete or coalescent granulomas, histologically characterized as noncaseating, are found. It was first described in 1875 by Jonathan Hutchinson and became known as “Mortimer’s malady,” after the name of the patient.’ Subsequently, in 1899, it was given the name sarcoidosis (“fleshlike”) by Boeck.’ The lungs are most commonly affected, presenting with the classic appearance of bilateral hilar lymphad- enopathy. Approximately 20% of patients are asymp- tomatic, with the disease discovered on routine radio- graphs.j Although all organ systems can be affected, the eyes, skin, and salivary gland involvement are most common. Manifestations in the oral and maxillofacial region are rare. Only 11 cases of sarcoidosis involving the maxillofacial skeleton have been reported in the literature.+‘+ We present a case involving the anterior maxilla and antrum.

Report of Case

A 40-year-old black man presented to the oral and maxillo- facial surgery clinic at the Atlanta Department of Veterans Affairs in June 1997 complaining of loosening of his front

‘Former Intern. Department of Oral and Maxillofacial Surgery, Emory University, Atlanta. GA; Current Resident, Department of Oral and Maxillofacial Surgery, University of Washington. School of Dentistry. Seattle. WA.

tChief, Department of OrAl and Maxillofacial Surgery. Depart- menf of Veterans mairs. Atlanta. GA.

Address correspondence and reprint requests to Dr Hong: Department of Oral and Maxiilofacial Surgery. [Jniversity of Washing- ton. School of Dentistry. Health Sciences Bldg. Rm 0241. Box 357134, Seattle, WA 98195; c-mail: jayhong@u.washington.edu o 2000 Amerlccn Associollon of Oral and Mox~lloiac~ol Surgeons

0278.2391/00/58040015$3 00/O

teeth of 2 months’ duration. He was asymptomatic except for transient episodes of mild discomfort.

The medical history was significant for sarcoidosis diag- nosed in 199 1. He had a history of skin lesions on both legs, which were examined by biopsy and found to be consistent with the diagnosis of sarcoidosis. He was initially placed on a regimen of prednisone, 40 mg daily X 30 days. The patient was taking plaquenil, 400 mg. He had also been diagnosed with chronic sinusitis in 1997, for which he has been treated with Augmentin (SmithKline Beecham Pharmaceuticals, Philadelphia, PA).

Extraoral examination showed a l-cm-diameter, purplish nodule in the right infraorbital region (Fig 1). The lesion was of 2 months’ duration and was nontender. He had a similar nodule in the left deltoid region (Fig 2). The patient related that the skin lesions were identical to the ones that had biopsies done in 199 1.

Intraoral examination showed significantly mobile maxil- lary right lateral and right and left central incisors. AI1 of the involved teeth tested vital to electronic pulp testing and were nontender. There was no expansion of the anterior maxilla, but the buccal gingiva felt soft and rubbery, and no buccal cortical plate could be detected.

Radiographic studies, consisting of panoramic (Fig 3) and occlusal radiographs (Fig 4). showed a diffuse, welldelined ndiolucency involving the anterior maxilla and hard palate. A minimal trabecular pattern could be seen in the ndiolu- cency, and there appeared to be a loss of the lamina dura around the involved teeth. Serial computed tomography scans showed a radiodense lining of the maxillary sinus and erosion of the nasal floor (Fig 5).

An incisional biopsy was made in the anterior maxilla and submitted for microscopic examination. The specimen showed a noncaseating granuloma with aggregates of giant cells and epithelioid histiocytes that was highly suggestive of sarcoidosis (Fig 6).

After a review of the literature and consultation with the patient’s puhnonologist, it was decided to attempt a conser- vative approach initially. A splint was constructed with orthodontic arch wire and composite bonding agent extend- ing from the maxillary right canine to the maxillary left canine. Because the patient had a preexisting open bite, there were no concerns about traumatic occlusion on the mobile teeth. A tapering regimen of prednisone was initi- ated by the puhnonologist, starting with a dose of 40 mg

436 INTRAOSSEOUS SARCOIDOSIS OF THE MAXILLA

FIGURE 1. Purplish nodule 1 cm in diameter in right infraarbital region. A biopsy wos performed on on identical lesion with a diagnosis of sarcoidosis from the left leg 5 years eorlier.

daily. The patient was then placed on follow-up every 4 weeks. At the’ s-month follow-up visit, the arch wire was removed, and the teeth were stable. The radiograph showed some increased trabeculation (Fig 7). Electronic pulp testing showed all the involved teeth to be vital.

Discussion Many theories have been proposed for the cause of

sarcoidosis, from infectious to immunologic. It is now accepted that the cause is multifactorial. Women are more commonly affected than men, and the preva- lence among blacks is 10 times higher than in whites. The clinical course in blacks also has a poorer progno- sis. The condition is most common in the second and fourth decades, but it can affect individuals of any age.r5

Sarcoidosis is usually self-limiting, with a fluctuating course of exacerbation of the clinical symptoms. Common symptoms involving the pulmonary system include dry cough and dyspnea, along with fever, lethargy, fatigue, and malaise. The skin is involved in up to 25% of cases. The lesions are characterized by

FIGURE 2. Nodule in left deltoid region.

FIGURE 3. Panoramic radiograph showing the radiolucency in the onterior maxillary region.

nontender, indurated, dark purple, skin plaques on the limbs, back, and buttocks, known as erythema nodosum. However, they are not pathognomonic for sarcoidosis, because other systemic diseases also can have this manifestation. When the skin lesions appear as symmetrical, infiltrative, violaceous plaques on the nose, cheek, ears, forehead, and hands, the condition is known as lupus pemio.

FIGURE 6. A, Low- and 6, medium-power photomicrogra hs show ing the noncaseoting gronulomatous tissue and epithelioid rstrocytes r. (H&E, original magnifications x40 and x 100).

HONG AND FARISH

FIGURE 4. Occlusal radiograph shawin the extensive bony destruc- tion of the anterior maxilla. Nate the lass a lamrna dura. 9

Ocular involvement is not uncommon, with inflam- mation of the anterior uveal tract or secondary glaucoma. Keratoconjunctivitis sicca is due to involve- ment of the lacrimal glands. When this is associated with parotid gland enlargement, it is also known as uveoparotid fever or Heerfordt’s syndrome. Lof- gren’s syndrome is delined as the triad of ery- thema nodosum, bilateral hilar lymphadenopathy, and arthralgia.

Oral involvement in sarcoidosis is uncommon, and it is most often seen as a soft tissue lesion. Blinder et alI6 reported 33 welldocumented cases of soft tissue sarcoidosis, of which 10 of the cases were located in the buccal mucosa and vestibule. These soft tissue

FIGURE 5. Coronal CT scan showing radiapacity of the left maxillary sinus and destruction of the nasal floor.

FIGURE 7. Three-month follow-up radiograph showing increased trabeculation in the anterior maxilla. Some of the integrity of the lamina dura has been regained.

lesions are clinically identical to the granulomas seen in systemic inflammatory diseases such as Crohn’s disease or infectious processes such as tuberculosis or actinomycosis. Furthermore, fungal infections, such as blastomycosis and histoplasmosis, also must be ruled out. Salivary gland involvement presents as an expansile parenchymal mass,17.*8 similar to lesions also seen in lymphoma, Sjogren’s syndrome, and metabolic conditions such as diabetes mellitus, malnu- trition, and alcoholism. Tongue lesions appear as broad, indurated, elevated masses. *%2o Gingival involve- ment is characterized by erythema of the papillae and attached gingiva, similar to what is seen in drug- induced gingivitis or plasma cell gingivitis, frequently associated with nodular soft tissue masses on the attached mucosa.2s21 Asymptomatic, expansile lesions in the floor of the mouth can be easily misdiagnosed as a mucocele or ranula.22-24 Palatal lesions appear as multiple, small, submucosal nodules resembling an abscess or tumor.8J2*20 Cahn et al2s performed biop sies on 23 patients with known sarcoidosis and found that palatal biopsy specimens of clinically normal areas showed sarcoid granulomas in 38% of the samples.

Osseous involvement in sarcoidosis is rare and, when present, usually involves the distal phalanges, causing punched-out radiolucent lesions that are well corticated. Involvement of the bony skeleton of the face is extremely rare.

The diagnosis of sarcoidosis is one of exclusion. A gold standard diagnostic modality for determining the activity and progression of the disease is lacking. The traditional indicator of activity is based on the radio

438

graphic appearance on the chest radiograph?

Stage I: Bilateral enlargement of the hilar nodes with- out pathologic changes in the lung fields (60% to 80% remission rate)

Stage II: Bilateral enlargement of the hilar lymph nodes with pathologic changes in the lung fields around the hili (60% remission rate)

Stage III: No enlargement of the lymph nodes, but extended, sometimes patchy or stripped, changes in both lung fields. The patches can join together, and bullae may form (<30% remission rate)

The serum angiotensin-converting enzyme (SACE) level is the most widely used laboratory test, with a spec- ificity of 90%.26 Other adjunctive tests include pulmo- nary function tests (namely, FEY, and vital capacity), gallium67 scintigraphy, and serum calcium levels. Bron- choalveolar lavage has also shown to be predictive of short-term outcome.26 The Kveim-Slitzbach test has been used extensively for diagnosis. It consists of a spleen extract from a known sarcoid lesion that is injected intra- dermally. A positive test results when a nodule devel- ops in 4 to 6 weeks. A biopsy of the nodule is done for confirmation. Because of the low specificity and sensitivity, the test is now mostly of historical interest.

Up to 60% of patients do not require treatment, because spontaneous resolution occurs in a significant number within 2 years. I5 Another 20% with the more aggressive forms are treated with corticosteroids. ChIoroquine has also been useful alone or in combina- tion with steroids. The general prognosis is good, but continuous monitoring with chest radiographs and SACE levels are needed.

There are 11 documented cases of involvement of the jaws: 5 in the maxilla, 4 in the mandible, 1 in the condyle, and 1 case involving both jaws’” (Table 1). The bony lesions involving the mandible appeared to be

mainly focaI, periapical ndiolucencies involving the roots of the posterior teeth. More extensive lesions were seen in the maxilla, frequently in the anterior maxilla, involving the incisors, nasal floor, and sinuses. One case was reported in the right condyle that was treated with a high condylectomy.‘3 However, it was not clear whether the lesion was truly sarcoid or secondary to chronic steroid use in treatment of the systemic lesions.

All cases were microscopically confirmed with the diagnosis of sarcoid granuloma. Because of the ex- treme paucity of these cases, a definite treatment plan has not been established. Because of the waxing and waning nature of the disease. complete resolution can occur without any interventionx

Aragon et al’” and Rubin et alI3 described involve- ment of the anterior maxilla with osseous Iytic lesions and mobile maxillary incisors. Treatment consisted of extraction of anterior maxillary teeth, with complete resolution of lesion. Betten and Koppang’ reported a sarcoid lesion presenting as a radiolucency of the apex of the mandibular first premolar. The patient was treated with an apicoectomy, and complete osseous regeneration was noted at the 11-month follow-up. Multiple reports of lytic lesions in the edentulous maxilla or mandible have been described by Kalman and Mallett,” Cohen and Reinhardt,’ Hillerup,6 and MacDonald et al.” AII were treated with surgical currettage, with excellent long-term results. Reports by Hildebrand et alI2 and KIesper et alI4 showed resolution of the lesions with conservative medical treatment with short-term regimens of prednisone, 10 to 40 mg daily. These case reports involved extensive lesions that would not have been feasible to treat surgically. All of the patients in the reported cases with intraosseous lesions had a previous diagnosis of sarcoidosis based on extraoral involvement, except for 1 case in which the oral involvement was the initial presenting symptom.- This is in contrast to the 33 soft

Table 1. DOCUMENTED CASES OF INTRAOSSEOUS SARCOIDOSIS IN THE JAWS

AS Name o’r) Chief Complaint Location Treatment

Aragon et al”) 25 Sore gums Anterior maxilla Extraction Hildebrand et al” 41 Nasal obstruction Anterior maxilla Steroids Kalman and Mallett” 54 Previous ext site Anterior maxilla Currettage Klesper et ali” 16 Loose teeth Right maxilla Steroids Rubin et ali> 25 Routine extraction Anterior maxilla Extraction Betten and Koppang- 40 Radiolucency mandibular right 1st Mandibular right 1st premolar Apicoectomy

premolar Cohen and ReinhardtO 59 Nonhealing ext Exterior site mandibular left 2nd Currettage

premolar Hillerup6 22 Previous ext site Right mandible Currettage MacDonald et al” 40 Edentulous Bilateral mandible Currettage Thomas et al5 30 Pain right face Right TMJ High condylectomy Cohen et al* 35 Asymptomatic Right mandible Observation

GADRE AND ZliBAlR\

tissue sarcoid cases reported by Blinder et al,‘” in which 16 of the patients had no previously known diagnosis of sarcoidosis.

Although the data are scarce, it has been suggested that intraoral sarcoid lesions are not as rare as reported in the literature. Therefore, intraoral lesions causing asymptomatic swelling should give rise to the suspi- cion of sarcoidosis, and it should be included in the differential diagnosis, especially if the patient has had a previous diagnosis of systemic involvement. Even though the patient in this case report showed some resolution of lesions through conservative treatment, such treatment cannot be recommended for all cases of intraoral sarcoidosis without proper evaluation of the activity and progression of the disease.

References 1. Kerr NW: Sdrcoidosis. Oml Surg 20: 166. 1965 2. Kolas S. Rochr WC: Sarcoidosis lesions primar) in the otxl

cavitj? Report of a cast. J OrdI Surg 18: 169. 1960 3. Harrison TR. Fauci AS: Principles of Intrmal Medicine (ed 1-t).

New York. NY. McGrdw-Hill. 1998. p 1923.1928 -1. Kalman SI. Mallrtt SF’: Aherrdnt gland and sarcoidosis in the

maxillae: Report of a case. J Oral Surg 12:63, 195-t 5. Thomas RF, ~Mcrkow L. White NS: Sarcoidosis with in\olvement

of the mandibular condyle. J Ordl Surg 3-t: 1026. IV76 6. Hillcrup 5, Diagnosis of sarcoidosis from ordI manifestation. Int

J Oral slug 595. IT6 7. Betten B. Koppang HS: Sarcoidosis with mandibular involvc-

ment: Report of a case. J Oral Surg 42:73 1, 1976 8. Cohen C. Krutchkoff D. Eisenherg E: Systemic sarcoidosis:

Report of two cases with oral lesions. J Otxl Surg 39:613. 1981

j Oral Max~llofoc Surg 58 439-443 2000

9.

IO.

11.

12

13.

14.

15.

16.

17.

18.

19.

20.

21.

22.

23.

2-1.

Cohen DM. Reinhardt RA: Systemic sarcoidosis presenting with Homer’s syndrome and mandibular paresthesia. Or,ll Surg 53:5”. 1982 Ardgon SB. Coke JM. Greer RO: Sarcoidosis with involvement of the maxillx J Oral .Med 3’:52. 1982 MacDonald DG. Rowan RM. Blair GS: Sarcoidosis involving the mandible: A cast report. Br Dent J 126: 168. 1985 Hildehrand J. Plezia Rh. Rao SB: Sarcoidosis: Rcpon of two cases with oral involvement. OrdI Surg 69:21’. 1990 Ruhin M. bnfilippo R. Pliskin A: Maxillary alveolar hone loss in a pdticnt with sdrcoidosis. J OrdI Maxillopdc Surg 49: 1351. 1991 Klesper B. Schmclzlc R. Donath K: Cutaneous manifestation of sxcoidosis with severe osseous destruction of the midface: A case report. J Crdnioma.. Surg 22: 163. 199-1 Neville BW. Waldron CA, Hers&r& EE: OraI and Maxillobcial Pathology (cd 1). Philadelphia. PA, Saunders. 1995. pp 241-243 Blinder D. Yahatom R. Taichcr S: Oral manifestations of sarcoidosis. Ordl Surg 83:458. 1997 Hogpins GS. AIlan D: Sarcoidosis of the maxillary region. Oral Surg L&623. 1969 Gold RS. Sager E: Oral sarcoidosis: Review of the Iiterdture. J Otxl Surg 34:23-. 1976 Tillman HH. Taylor RC, Carchidi JE: Sarcoidosis of the tongue. OralSurg21:190. 1966 Van Madrsseveen ACM, Van der Waal 1. Stam J. et al: Ordl involvement in sarcoidosis. Int J Oral Surg 11:21. 1982 Hobkirk JA: Sdrcoidosis with oral lesions: Report of case. J Oral Surg 27:89 1, 1969 Orlean SL. O’Brien JJ: Sarcoidosis manifesting a soft lesion in the floor of the mouth. OraI Surg 21:819. 1966 Roche WC, Morris CR, Nemickas R: Sdrcoidosis of sublingual gkmds: Report of a case. J Oral Surg 25:77. 1967 Nardng R. Dixon RA Jr: Sarcoidosis and rdnula of a sublingual gland. Oral Sug 39:376. 1975

25. Cahn LR. Eisenhud L. Blake MN, et aI: Biopsies of norm;ll- appedring palates of patients with known sdrcoidosis. Oral Surg 18:3-&L. 1964

26. Consensus conference: Activity of sdrcoidosis. Third WASOG meeting. Los Angek~;. CA. September Kl 1. Eur RespirJ 7:624, 199-i

Aneurysmal Bone Cyst of the Mandibular Condyle: Report of a Case

K.S. Gadre, MD& * and R.A. ZuDai)y, Mchf

Benign and malignant tumors of the temporomandibu- lar joint are rare.’ An aneurysmal bone cyst (ABC) of the condyle is even more unusual, with only 2 cases reported in the literature.l.3 ABC is an unusual bone lesion, affecting mainly the long bones and only rarely the facial bones:’ The clinical features are ordinarily

‘Maxillofacial Consultant, Ruby Hall Clinic. Pune. India.

tConsultant Pediatric Surgery, Ruby Hall Clinic, Pune. India

Address correspondence and reprint requests to Dr Cadre:

Sunrise, Vijapnagdr colony. Behind Kaveri Hotel, Pune 41 I-030. MS,

India; e-mail: kiran_gadre@hotmaiI.com

o 2000 American Assoc~ot~on of Oral and Moxlllofoctol Surgeons

0278.2391/00/58040016$3 00/O

439

mild,5 and most commonly the lesion appears as slowly expanding growth,” causing facial asym- metry because of the “ballooned out” appearance for which the lesion was originally named. Jtie and Lichtenstein’.x in 1942 used the term aneurysmal cyst, and in 1950 they used the term aneurysmal bone cyst.

Fifty percent of ABCs arise in the long bones, and 20% occur in the vertebral column. ABC accounts for 1.5% of the nonodontogenic, nonepithelial cysts of the mandible.Y,10 It occurs in persons younger than age 20 years, and, according to Ariel et al,” the mean age-group with skull and facial lesions is 14.3 years. A history of trauma often precedes development of the lesion.12,13 Eveson et alI+ maintain that a history of