562 POSTEST~CTION PANFACIAL CELLULITIS

Other diseases, cysts, ameloblastoma, osteomyelitis, aneursymal bone cyst, giant cell tumor, and benign and malignant cartilaginous tumors, also may show similar signs,‘&l~ A biopsy is required to show the histologic diagnosis.‘,”

Secondary inflammation in the underlying bone in very common and may give rise to general symptoms, such as fever, asthenia, and headaches.” In this case, no such complications were present.

Treatment of eosinophilic granuloma is by surgical curettage, excision, or radiation therapy.2,i,--‘1,13 In this case, condylectomy as an excisional biopsy was performed. Irrespective of the treatment, the recur- rence rate is low.” However, the patient in this case will be closely followed-up for an extended period.

References 1. Lichtenstein L: Integration of eosinophilic granuloma of hone.

“Letterer-Siwe” disease and “Schullcr-Christi:tn” disease as related manifestations of a single nosothologic entiw. Arch Path01 56:84. 1953

2. Wong GB. Pharoah hlJ, Weinberg S. et al: Eosinophilic grJn”-

3.

4.

5.

6.

x.

9.

10

11.

12.

I.3

loma of the mandibular condyle: Report of three cases and review of the literature. J Oral Maxillofac Surg 55:8-O. 1997 Roychoudhur) A. Shah N. Parkash H. et al: Eosinophilic granuloma of the jaws. Br J Oral IMzxillofac Surg 36:3BO. 199R Cmnda FM, McDaniel RK: Multiple progressive eosinophilic granuloma of the jaws. J Oral Maxiilofac Surg 10:171, 1982 Sakashita H. ,Miyata M. jkliyamoto H. et al: Unifocal eosinophilic gmnuloma of the buccal gland: Reporr of a case. J Jpn Stomatol soc41:119. 1992 Hartman KS. Colonel L: Histiocytosis X: A review of 11-i cases with oral involvement. Oral Surg Oml Med Oral Path01 i9:38. 1980 Bhaskar PD. White CS. Baughman RA: Eosinophilic gnnuloma of the mandibular condyle: A case report and management discussion. Oral Surg Oml Med Oral Pathol76:557. 1993 Peckitt NS. Wood GA: Eosiniphilic granuloma of the mandibular condyle. BrJ Or4 Maxillofacial Surg 26:306. 1988 Makek hl. Sailer HF: Eosiniphilic granuloma of the mandibular condyle: Cdsc- report J Masillofac Surg 8:327. 1980 Tant L. Dourov N. Vanatoru P, et al: Eosinophilic grznuloma (histiocytosis X): Apropos of a case located at the level of the mandibular condyle. Acta Stomatol Belg Hl: 181, 198-1 jr;ichariades N. Anast;tse;i-Vlachou K. Xypolyta A, et al: Uncom- mon manifestations of histiocytosis X. Int J Oral iMasillofac Surg 16355. 198’ Damantc JH. Alvares LC. Chinellato LEM: Eosiniphilic gram,- loma of mandible. Oral Surg Oral Med Oral Pathol 5 1 :-li6. 1981 Whitcher BL. Webb QJ: Treatment of recurrent cosinophilic granuloma of the mandible following radiation therapy. J Oral Maxillr)Etc Surg -r-1:565. 1986

J Oral Max,llofac Surg 58 562-566. 2000

Postextraction Panfacial Cellulitis (Sweet’s Syndrome) Mimicking

an Odontogenic Infection Martin Steiner, DDS, * Alan R. Go~dd, DDS, MS, f

Paul Jeffy Brooks, DDS, MD,+ and Kerlit? Porter, DDS, MDJ

Facial cellulitis of odontogenic origin is a common occurrence but is rare when attributable to noninfec- tious cause. In 1964, R.D. Sweet, a British dermatolo-

‘Professor, Department of Surgical/Hospital Dentistry. School of

Dentistry, University of LouisviUe. Louisville. KY.

tprofessor, Depanment of Surgical/Hospital Dentistry, School of

Dentistry, University of Louisville. Louisville. KY.

*Former Chief Resident, Department of Surgical/Hospital Den- tistry, School of Dentistry. University of Louisville, Louisville. KY:

Currently in Private Practice. Grand Rapids. MI.

IFormer Chief Resident. Department of Surgical/Hospital Den- tistry, University of Louisville. Louisville, KY: Currently in Private

Practice, Odessa, TX.

Address correspondence and reprint requests to Dr Steiner:

Department of Surgical/Hospital Dentistry, University of Louisville.

KY 40292.

0 2ooO Amerlcon Assoc~ot~on of Oral and Max~llofoc~ol Surgeons

0278-2391 /OO/5805UCIl6$3 00/O

doI IO 1 053/lo 2000 5537

gist, described the findings in 8 patients with skin eruptions resembling erythema multiforme.’ Involve- ment of the facial skin was included. Histologically, dense dermal infiltrates of mature neutrophils were present. In a second report, Sweet2 termed this disorder “acute febrile neutrophilic dermatosis,” and this clinical entity has commonly been referred to as Sweet’s syndrome (SS). Subsequent reports have de- scribed this syndrome as characterized by erythema- tous facial lesions associated with cellulitis.+(’ Addi- tional significant clinical features of Sweet’s syndrome include fever, neutrophilia, and painful erythematous skin lesions that show a rapid response to corticoste- roid therapy. Eighty percent of cases are idiopathic, 17% are associated with a hematologic malignancy, and 3% are encountered in patients with genitourinary malignancy.’ A literature review disclosed nearly 500 published cases of SS in the English language medical literature, with most in the dermatology literature. A significant number of cases were also found in the Japanese literature. However, only a single case report

STEINER ET AL

appears in the dental literature, that being a patient with oral cancer undergoing radiation therapy with subsequent development of skin and facial lesions about the oral mucosa and lips characteristic of Sweet’s syndrome.8

It has been estimated that between 10% and 20% of cases of Sweet’s syndrome occur in association with malignancy, with the most common malignancy being acute myelogenous leukemia.9 We report a patient initially admitted to the hospital for management of acute facial cellulitis of suspected bacterial origin. Subsequent studies disclosed acute myelogenous leu- kemia and Sweet’s syndrome.

Report of Case

An l&year-old white man underwent elective extraction of an impacted tooth lower left third molar at the Oral and Maxillofacial Surgery Clinic of the University of Louisville, without apparent complications. At the time of surgery, there was no local evidence of oral inflammation and infection. The initial recovery period was uneventful, but 4 weeks postoperatively, he returned with left submandibular swelling. Clinical examination indicated moderate swelling in the mandibular buccal sulcus in the left second and third molar area. The patient was afebrile and in no acute distress. An intraoral incision and drainage was performed, yielding only a few milliliters of pus. A Penrose drain was placed, penicillin V (500-mg capsules 4 times daily) and Flagyl (Searle, Chicago, IL; 500 mg capsules 4 times daily) were prescribed, and the patient was instructed to return the following day. That evening, the patient reported to the

563

University of LouisviIle Hospital emergency room with an oral temperature of 101.9”F, increased facial swelling, and local erythema. The swelling and erythema extended from the left submandibular area to the left periorbital and left temporal regions (Fig 1). The erythema had also spread to the right periorbital area, giving the overall appearance of a panfacial cellulitis. A complete blood examination indicated a leukocyte count of 6,6003, hemoglobin of 8.3 g/dL hematocrit of 24.4%, and a platelet count of 188,0003. The differential cell count was: 4% granulocytes; 62% lymphc- cytes; 12% monocytes; and 22% blast cells. Because of the decreased granulocyte and elevated blast cell counts, a consultation with the Medicine and Hematology/Oncology services was requested. Intravenous antibiotic, consisting of 3.0 g Unasyn (Roerig, New York, NY; every 6 hours), was begun, and a head and neck computed tomography (CT) examination was obtained. Images of the head and neck indicated only soft tissue swelling, with neither discrete fluid collection nor abscess formation in the left temporal and left submandibular areas (Fig 2). Minimal deviation of the airway was observed.

On the presumption of a rapidly progressive panfacial bacteria1 cellulitis, it was deemed necessary to take the patient to the operating room to create incisions for local decompression and to provide pathways for drainage of pus. A fiberoptic technique for intubation was successfully performed, although it was fairly traumatic. Extraoral inci- sion and drainage were performed in the left submandibular space region and several drains placed. Again, a few millili- ters of pus were obtained, and the material was submitted for aerobic and anaerobic cultures. Because of the trauma associated with intubation, an immediate dose of 10 mg Decadron (Merck, West Point, PA) was administered intrave- nously to reduce laryngeal edema and maintain the airway, and a second dose given 4 hours subsequently.

FIGURE 1. Frontal and oblique views of the otient. A Swelling and erythemo extending from the left submondibulor Oreo to the left periorbitol and left temporal areo. 6, Fociol swelling with eryt

R .‘. emo in the right and left periorbitol oreos.

564 POSTEXTRACTION PANFACLAL CELLULITIS

FIGURE 2. CT scan showing soft tissue swelling in leh mossetenec and buccol regions without fluid collection.

The following morning, a marked reduction in facial erythema and left temporal edema was observed. Blood cultures were negative, and the intraoperative cultures Indicated a 3+ growth of Staphylococcous aureus. Two grams intravenous cefepime was administered every 12 hours. SweIIing and Induration of the left neck were slow to resolve.

A bone marrow sample was obtained for laboratory analysis. Wright-Giemsa-stained cytospin preparation re- vealed the bone marrow essentially replaced by blasts. Although rare azurophilic granules were observed, no Auer rods were identified. Approximately 5% of blasts were myelopeioxidase positive, with no blast cells showing nonspecific esterase positivity. lmmunophenotyping showed a vast predominance (93%) of blast cells with the positive immunophenotype consisting of dim CD45, CDl3, CD34, CD7 and HLA-Dr. These findings were interpreted as indicat- ing acute myelogenous leukemia, subtype Ml (French- American-British [FAB]) group classification.‘9 The patient was transferred to the Hematology/Oncology Service for definitive treatment. ldarubicin and arabinosylcytosine were used during the first cycle of chemotherapy. The patient had a stormy remission Induction course because of continuous submandibular drainage, progressing to a methicillin- resistant S aureus septicemia. In addition, creatinine levels rose to 2.3 mg/dL, an indication of acute renal failure considered secondary to the immunocompromised status, chemotherapy, and sepsis. He received granulocyte transfu- sions, which Improved his blood picture. AII complications associated with chemotherapy ultimately resolved, and he entered his first remission. A subsequent bone marrow aspirate showed no evidence of leukemic disease.

Discussion

Su and L.iu” have proposed major and minor diagnostic criteria for SS. Major criteria are 1) abrupt onset of tender, painful erythematous or violaceous plaques or nodules; and 2) a predominantly neutro- philic infiltration in the dermis without leukocytoclas- tic vasculitis. Four minor diagnostic criteria include 1)

illness preceded by fever or infection; 2) illness accompanied by fever, arthmlgia, conjunctivitis, or underlying malignancy; 3) leukocytosis; 4) a prompt response to corticosteroid therapy, but little response to antibiotics. They suggested that a definitive diagno- sis of SS should be made when both major criteria and at least 2 minor criteria are fulfilled. Von den Driesch et al” reviewed 6 cases of SS, finding that all of their patients had an erythrocyte sedimentation rate above 50 mm per hour. These authors suggested that a sedimentation rate elevated to at least this value should be considered a minor diagnostic criterion. A sedimentation rate was not obtained in the current case.

Fitzgerald et al’” stated that anemia and an abnormal platelet count are commonly encountered in patients with malignancy-associated SS. In the current case, the patient had a history of a very low hemoglobin level since childhood, which remained unexplained after medical workup. On admission, his hemoglobin was 8.3 g/dL. Of the major criteria, this patient had erythematous plaques on his face (Fig 2). Because of the presence of acute myelogenous leukemia and agranulocytosis, a very low hemoglobin of 8.1 g/dL and acute renal failure, a cutaneous biopsy for confir- mation of SS was not performed. Of the minor criteria, this patient had fever, arthralgia, a low hemoglobin level, infection, malignancy, and a very good response to corticosteroid therapy. Cohen and Kurzrock” stated that most patients with SS show all the criteria of this entity, yet fever and an elevated circulating neutrophil count may be absent in some patients with biopsy- confirmed malignancy associated with SS. Probert et alI4 stated that SS should be considered when patients with acute myelogenous leukemia clinically develop characteristic inflammatory skin lesions and an unex- plained fever. Kemmett and HunterI reviewed 29 cases of SS and observed that although 1 or more of Sweet’s original diagnostic criteria were absent, an underlying disease was present in more than 50% of their cases.

In the current case, a provisional diagnosis of bacterial facial cellulitis as a postoperative complica- tion of removal of an impacted lower left third molar was initially made. However, the clinical findings were not fully consistent with an infectious process. Periorbital edema on the right side of the face, a low white blood count with an acute facial cellulitis, plaque-like nodules in the facial skin, and dramatic resolution of the erythema in the right periorbital area and left temporal edema within 24 hours of initiation of corticosteroid therapy appeared inconsistent with a diagnosis of facial cellulitis of bacterial origin. The bone marrow aspiration findings disclosed acute my- elogenous leukemia, raising the possibility of a cutane- ous paraneoplastic disorder. A consultation with the

STEINER ET AL

Dermatology Service of the LJniversity of Louisville Hospital indicated that there was more than adequate presumptive evidence to support the diagnosis of malignancy-associated SS.

Several excellent review articlesi~~r(‘~r- on SS appear in the literature, with Cohen et al’- specifically discuss- ing malignancy-associated SS. Su et alrx outlined the diagnostic criteria to permit separation of SS from clinically similar dermatologic conditions. SS shares features with erythema multiforme, because both may present with abrupt onset of clinically heterogenous cutaneous disease showing prominent erythema. In distinction to erythema multifomle. SS does not pro- duce typical target lesions, Erythema nodosum also can present with nodules and plaques, yet the ana- tomic distribution is usually limited to the lower extremities. Other conditions showing some clinical similarity to SS include erythema perstans. erythema elevatum, pyoderma gangrenosum, and leukocytoclas- tic vasculitis. Bromoderma and iododerma represent inflammatory diseases in response to bromine- and iodine-containing materials, respectively. Although these conditions show clinical and histologic similari- ties to SS. a history of ingestion of these compounds serves to distinguish them.

Episodes of SS may show a duration of 1 to 2 months. Ix The cause of SS is unknown; however, association with upper respiratory tract infection, inflammatory bowel disease, rheumatoid arthritis, sar- coidosis, vaccination, and pregnancy have been re- ported.“’ A minor odontogenic infection may have played a precipitating or contributing role in the current case. The pathogenesis of SS is also unknown, but it is believed to be attributable to hypersensitivity and immunologic phenomena.‘” Going’” stated that the cytokine interleukin-1 may play a central role in SS as an inflammatory mediator responsible for many of the clinical signs and symptoms.

The treatment of choice for SS is systemic corticos- teroids, usually consisting of oral prednisone at a dosage of 30 to 60 mg/d.lX Additional successful therapies include systemic administration of potas- sium iodide,” colchicine.‘~ and daps0ne.l Iodides influence mast cell behavior, resulting in reduced intensity of clinical signs and symptoms in hypersensi- tivity reactions.” Colchicine may be effective by inhibiting polymorphonuclear cell chemotaxis.?l and dapsone efficacy may be based on inhibition of lyso- somal enzyme re1ease.l

Wilson and Lynchl” stated that the malignancy most commonly associated with SS is acute myelogenous leukemia. As in the current case, onset may coincide with discovery of the malignancy. Alternatively, the syndrome may precede or follow the discovery of a malignancy. Cohen et all- reviewed 69 patients with hematofogic malignancy-associated SS. They found

56.5

42% of the malignancies were acute myelogenous leukemia. Other malignancies included lymphoma, chronic leukemia, myeloma, and myelodysplastic syn- dromes. Their findings indicated that onset of SS either preceded or coincided with the discovery of a previously undiagnosed cancer in 60% of the cases. Malignancy-associated SS tended to produce greater severity of cutaneous lesions than idiopathic SS.

Cohen et aP reviewed 39 patients with solid tumor malignancies that had SS. The most common malignan- cies originated in the genitourinary tract (37X), breast (23%) and gastrointestinal tract (17%) with adenocar- cinema constituting 57% and squamous cell carci- noma 11% of this group. Four of the patients with solid tumor-associated SS also showed concurrent hematologic malignancy. The authors reported that cutaneous symptoms occasionally resolved spontane- ously, and that corticosteroid therapy produce a salutory response and relief from some symptoms in hours and resolution of erythema and pain in 1 to 2 days.

Many patients with SS have shown facial skin involvement. Dompmartin et al” described a 3Gyear- old patient with an atypical facial erysipelas who was diagnosed with acute myelomonocytic leukemia 2 months previously. This case showed considerable similarity to the presently reported case, with ery- thematous, tense, warm edema of both cheeks and eyelids responsive to systemic corticosteroids. Terce- dor et aI3 reported a 73-year-old patient with an erythematous halo on the left ala of the nose and erythematous lesions on the left side of the face. Subsequent laboratory studies and bone marrow aspi- ration indicated acute myelogenous leukemia, support- ing the diagnosis of SS. The authors concluded that SS should be considered in the differential diagnosis when cutaneous facial lesions in febrile patients with leukemia are present, regardless of the peripheral leukocyte count and the appearance of the cutaneous lesions. Krilov et al’ reported a 3.5-year-old child presenting with facial cellulitis and juvenile chronic myelogenous leukemia. Labial and cutaneous cheek lesions were described, with later involvement of the forehead. The patient was treated with methylpredniso- lone after a diagnosis of SS was made, and the edematous areas resolved in 3 days. Bulengo-Ransby et al5 described a do-year-old man who had a rapidly spreading facial eruption that was preceded by respira- tory infection and arthralgia. Biopsy of the facial skin showed extensive dermal neutrophilic infiltrates, con- firming the diagnosis of SS. The authors reported a second case of a 57-year-old with similar lesions. Both patients responded to prednisone therapy, with nei- ther patient showing evidence of underlying malig- nant disease. Driban and Alvarez” described the oral manifestations of SS, including erosive lesions of the

566 POSTEXTRACTION PANFACLAL CELLlILITIS

buccal mucosa and fibrinous exudates and peripheral welldemarcated erythema. Similar lesions of the oro- pharynx, uvula, and tongue were described. Skin biopsy confirmed the diagnosis of SS, and the patient had a dramatic response to 60 mg prednisone daily. Bamelis et al26 presented a patient with SS and acute myelogenous leukemia exhibiting abrupt onset of massive tongue swelling. The authors suggested that the sudden macroglossia was produced by infiltration of mature neutrophils, an alteration encountered in several internal organs and mucosal sites of patients with SS. After 2 days of corticosteroid treatment, the tongue regained normal dimensions, and the cutane- ous lesions regressed. GimenezArnau et a12’ reported the first case of SS associated with multiple oral squamous cell carcinomas. Two months after effective therapy for SS, oral tumors were evident in the right soft palate and in the left side of the floor of the mouth. Biopsies showed both to be squamous cell carcinoma. Radiation therapy produced complete re- mission of the neoplastic lesions. However, 1 year later, SS recurred. Examination disclosed recurrence of the floor-of-the-mouth tumor and development of a new primary lesion in the retromolar area.

Based on the resemblence of SS to cellulitis of odontogenic origin, this disorder should be consid- ered in the differential diagnosis of an inflammatory facial swelling in which there are atypical clinical findings, and especially when there is a diagnosed malignancy.

References 1. Sweet RD: An acute febrile neutrophilic dermatosis. Br J

Dermatol76:349, 1964 2. Sweet RD: Further observations on acute febrile neutrophilic

dermatosis. Br J Detmatol80:800. 1968 3. Tercedor J. Rodenas JM. Hennz MT, et al: Facial cellulitis-like

Sweet’s syndrome in acute myelogenous leukemia. Int J Derma- to1 31:598, 1992

4. Krilov LR, Jacobson M. Shende A: Acute febrile neutrophilic dermatosis (Sweet’s syndrome) presenting as facial cellulitis in a child with juvenile chronic myelogenous leukemia. Pediatr Infect Dis 6:77, 1987

5. BulengcFRansby SM. Brown IMD. Dubin HV, et al: Sweet’s syndrome presenting as an unusual periorbital eruption. J Am Acad Dermatol24:140. 1991

6. Dompmartin A, Troussard X, Lorier E. et al: Sweet syndrome associated with acute myclogcnous Icukcmia: Atypical form simulating facial erysipclas. Int J Dermatol 30.6-i.l. 1991

-‘. Baer MR: livlanagemcnt of unusual prcscntations of acute leukemia. Hematol Oncol Clin North Am 7:2’5. 1903

8. van der Meij EH. Epstein JB. Hay J. ct al. Sweet’s syndrome in a patient with oral cancer associated with radiotherapy. Eur J Cancer B Oral Oncol 320: 133. 1996

9. Cohen PR. Kunrock R. Sweet‘s syndrome and cancer. Clin Dermatol 11: 149. 1993

10. Wiemik PH: Acute Icukemias. it1 Kelley WN (cds): Tcsth~x)k of Intcmal Medicine. Philadelphia. PA. Lippincott-Raven. 199’. p 1370

1 I. Su WFD. Liu HH: Diagnostic criteria for Sweet’s syndrome. Cutis 41:167. 1986

12. van den Dricsch P. Gomcz KS. Kicscwetter F, ct al: Sweet’s syndrome: Clinical spectrum and associated conditions. Cutis .44:193 1989 .

13. Fitzgerald RL. McBurncy El. Neshitt LT: Sweet’s syndrome. IntJ Dermatol 35:9, 1996

lr. Prohert C. Ehmann WC, Al-Mondhin’ H. et al: Sweet’s syndrome without gnnulocytosis. Int J Dermatol 3’: 108, 1998

15. Kcmmett D, Hunter JAA: Sweet’s syndrome: A clinicopatho- logic review of twenr)-nine cases. J Am Acad Dermatol 23:503. 1990

16. van den Driesch P: Sweet’s syndrome (acute febrile dermattr sis). J Am Acad Dcrmatol 31:535. 1994

17. Cohen PR. Talpaz M. Kurxock R: Malijin;tncy-;lssociated Sweet’s syndrome: Review of the world litemturc. J Clin Oncol 6- 188’. 1988

18. Su WPD. Fett DL. Gibson LE. et al: Sweet syndrome: Acute febrile neutrophilic dermatosis. Scmin Dermatol I+: I-3, 1995

19. Wilson BD. Lynch PJ’ Sweet’s syndrome and rclatcd disorders, in Sams WM. Lynch PJ (eds): Principles and Practice of Dermatology. New York. NY. Churchill Livingston. 1996. p 6: 1

20. Going JJ: Is the pathogcnesis of Sweet’s syndrome mrdiatcd b) intcrlrukin- I? Br J Dcrmatol 116:282. 1987

21. Horio T. Imamum S. Danno K. et al: Treatment of acute fchrilc neutrophilic dermatosis (Sweet’s syndrome) with potassium iodide. Dcrmatologica 160:3-i 1. I980

22. Suchisa S, Tagdnli H. Inouc F. et al: Colchicine in the treatment of acute febrile neutrophilic dermatosis (Sweet‘s syndrome). Br

.I debtor 108:99. 1983 23. Aram H: Acute febrile neutrophilic dcrmatosis (Sweet’s syn-

dromc): Response to Dapsone. Arch Drrmatol lLO:L-iS. 1984 2-i. Cohen PR. Holder WR. Tucker SB. et al: Sweet syndrome in

patients with solid tumor% Cancer ‘2:L723. 1993 25. Drihan NE, Alvarez MA: Oral manifestation of Sweet’s syn-

drome. Dermatologica 169: 102. 198~ 26. Bamelis M, Boyden B. Scnte F, ct al: Sweet’s syndrome and

acute myelogenous leukemia in a patient who presented with a sudden massive swelling of the tongue. Dermatology 190:335. 1995

27. Gimenez-Amau A. CarlesJ. Pla-Ferrer C. et al: Sweet’s syndrome and malignancy: A case associated with multiple oral squamous- cell carcinoma. Dermatology 193: 154. 1996