Recurrent facial abscess: Recurrent facial abscess: A rare presentation of pyoderma gangrenosumA rare presentation of pyoderma gangrenosum

Matthew C Mori, MD1; Margaret Carter, MD1

1Massachusetts Eye and Ear Infirmary, Harvard Department of Otology and Laryngology

INTRODUCTION NEUTROPHILIC DERMATOSES: PYODERMA GANGRENOSUM AND SWEET’S SYNDROME

WORKUPABSTRACT

CASE

REFERENCES

CONTACTCONTACT

Introduction: Nonhealing or recurrent facial abscesses or cellulitis are rare conditions that warrant further workup. Particularly in patients with inflammatory bowel disease, clinicians must be aware of neutrophilic dermatoses like pyoderma gangrenosum masquerading as infection.

Case: A 26 year old male presents with 6-week history of persistent worsening right-sided preauricular aseptic abscess now with a nonhealing draining wound. The patient underwent incision and drainage three times by Plastic Surgery and was treated with antibiotics with abscess reformation after each round of antibiotics. He had a history of ulcerative colitis and was having a flare on admission. On workup, he was diagnosed with pyoderma gangrenosum and began treatment with steroids and immunosuppressants. His swelling improved within 24 hours of beginning treatment and resolved after 1-2 weeks.

Discussion: This case illustrates the importance of considering neutrophilic dermatoses when treating aseptic, recurrent, or nonhealing facial abscesses or cellulitis or ulcers. Due to pathergy, multiple incision and drainage procedures can enlarge and worsen the abscess causing irreparable cosmetic damage. Appropriate treatment with steroids and immunosuppressants managed with Dermatology or Gastroenterology input can lead to rapid resolution.

• Cultures of thick yellow wound drainage No growth from three cultures sent for aerobic, anaerobic, & fungal

• CT: extensive facial cellulitis involving parotid with open wound.

• Dermatology consult: biopsy of arm nodule.

Figure 5. Deep dermal and periadnexal neutrophilic infiltrate. No microorganisms identified on Gram, AFB and PAS stains. Consistent with neutrophilic dermatoses.

Gastroenterology consult due to 7 week history of hematochezia with hx of UC. Underwent CT and flexible sigmoidoscopy with biopsies consistent with acute ulcerative colitis flare

DIAGNOSIS:PYODERMA GANGRENOSUM or

SWEET’S SYNDROMEin setting of ULCERATIVE COLITIS FLARE

Pyoderma gangrenosum Ulcerative skin disease typically begins as aseptic papule, pustule, or vesicle. TYPICAL (PG): Develops into painful ulcer with ragged, overhanging, dusky purple edges and surrounding induration and erythema. 80% lower extremities. <5% head and neck. ATYPICAL (APG): Superficial with hemorrhagic bullae. 77% upper extremities. Associated with hematologic malignancies ~50% Pathergy, lesions with skin trauma. Contraindication to aggressive debridement. Epidemiology: Rare. Occurring most frequently in young to middle-aged adults. F>M 50% systemic dz. >30% IBD (UC:Crohn’s 1:1). 20% arthritis. 27% APG heme malignancy Diagnosis of exclusion. Treatment: *Steroids, cyclosporine, TNF-αinhibitors (e.g. etanercept), dapsone

Sweet Syndrome (acute febrile neutrophilic dermatoses) fevers, tender erythematous plaques or nodules occur on face, neck, extremities Associated: URI/GI infections, IBD, pregnancy, hematologic malignancies Dermal neutrophilic infiltrate. NO vasculitis Treatment: Steroids, potassium iodide, colchicine, dapsone, chemotherapeutic agents

26-year-old male admitted to Otolaryngology-Head and Neck Surgery (ORL-HNS) service from ED due to recurrent facial abscesses and concern for purulent parotid fistula

• 6-weeks of persistent worsening right-sided preauricular facial abscess and cellulitis. Low-grade fevers

• Started as small pustule in preauricular area

•Underwent needle drainage and then incision and drainage (I&D) three times, first by PCP and then outside hospital Plastic Surgery (OSH PSY), of increasingly large abscesses.

•Underwent multiple rounds of antibiotics. However abscess would recur once off antibiotics. Wound failed to close.

•Past medical history significant for ulcerative colitis (UC) and ankylosing spondylitis. On review of systems endorsed hematochezia.

Facial abscesses and cellulitis are common problems otolaryngologists treat. Nonhealing facial abscesses and persistent or recurrent cellulitis are rare entities that warrant further thought and workup. In patients with inflammatory bowel disease (IBD), surgeons must be aware of neutrophilic dermatoses like pyoderma gangrenosum masquerading as infection. Surgical treatment of undiagnosed neutrophilic dermatoses may create a large nonhealing wound.

1. Andre MF, Piette JC, Kemeny JL, et al. Aseptic abscesses: a study of 30 patients with or without inflammatory bowel disease and review of the literature. Medicine (Baltimore). May 2007;86(3):145-161.

2. Bennett ML, Jackson JM, Jorizzo JL, Fleischer AB, Jr., White WL, Callen JP. Pyoderma gangrenosum. A comparison of typical and atypical forms with an emphasis on time to remission. Case review of 86 patients from 2 institutions. Medicine (Baltimore). Jan 2000;79(1):37-46.

3. Cohen PR. Neutrophilic dermatoses: a review of current treatment options. Am J Clin Dermatol. 2009;10(5):301-312.

4. Crum NF, Higginbottom PA, Fehl FC, Graham BS. Sweet's syndrome masquerading as facial cellulitis. Cutis. Jun 2003;71(6):469-472.

5. Harris AJ, Regan P, Burge S. Early diagnosis of pyoderma gangrenosum is important to prevent disfigurement. BMJ. Jan 3 1998;316(7124):52-53.

6. Pereira N, Brites MM, Goncalo M, Tellechea O, Figueiredo A. Pyoderma gangrenosum--a review of 24 cases observed over 10 years. Int J Dermatol. Aug 2013;52(8):938-945.

7. Surovy AM, Pelivani N, Hegyi I, Buettiker U, Beltraminelli H, Borradori L. Giant cellulitis-like Sweet Syndrome, a new variant of neutrophilic dermatosis. JAMA Dermatol. Jan 2013;149(1):79-83.

8. Travis S, Innes N, Davies MG, Daneshmend T, Hughes S. Sweet's syndrome: an unusual cutaneous feature of Crohn's disease or ulcerative colitis. The South West Gastroenterology Group. Eur J Gastroenterol Hepatol. Jul 1997;9(7):715-720.

9. Wittekindt C, Luers JC, Klussmann JP, Huttenbrink KB. Pyoderma gangrenosum in the head and neck. Arch Otolaryngol Head Neck Surg. Jan 2007;133(1):83-85.

Matthew C. Mori, M.D.Massachusetts Eye and Ear Infirmary243 Charles St, Boston, MA 02215Email: matthew_mori@meei.harvard.edu

EXAMINATION

Figure 1. Preauricular abscess after 1st I&D by PCP

Figure 2. Open wound after 3rd

I&D & debridement by OSH PSY

Figure 3 &4. On admission to ORL-HNS, preauricular draining wound with surrounding erythema. Arm nodule noted.

• Continued Antibiotics (Augmentin) for possible superinfection of open preauricular lesion. Cultures with no growth.•Started on high-dose pulsed steroids, initially Solu-medrol, then prednisone 60mg• Improved and discharged on hospital day 9.• Tapered off steroids over next 2-3 months• Wound granulated and healed.

POST-DIAGNOSIS COURSE

One year later• Presented with 3 days of rapid R facial swelling overlying mandible and large sterile abscess (Figure 6) in setting of prolonged UC flare.• After I&D at outside hospital, did not improve. Admitted to Medicine service and ORL-HNS consulted.• Again, improved on pulse steroids and antibiotics

Figure 6

Figure 7. Patient well-healed on most recent exam this year. Scars from each episode are visible: one in the preauricular

area and one overlying the mandible.

TAKE-HOME POINTS• Consider neutrophilic dermatoses (pyoderma gangrenosum or Sweet’s syndrome) in patients with aseptic, nonhealing, or recurrent facial abscesses; ulcers; or cellulitis. Especially in patients with IBD, heme malignancies, or RA.• If suspected, Avoid further surgical trauma, due to PATHERGY. • Primary treatment is steroids.

ACKNOWLEDGEMENTSACKNOWLEDGEMENTSORL-HNS team: Dan Fink, Blake Alkire, Jason Fowler, our patient.Brigham and Women’s Hospital Dermatology, Gastroenterology, & Pathology consultation services