Pathergy is a unique and dramatic clinical finding of exuberant inflammation in response to local trauma. This incompletely understood phenomenon presents with sterile pustules and ulcers after minor cuts or scrapes, and can be tested by a pricking the skin with a sterile needle. Historically, pathergy was thought to be pathognomonic for Behcet’s syndrome, though it has also been described in several other inflammatory conditions such as pyoderma gangrenosum and acute febrile neutrophilic dermatosis (Sweet’s syndrome). Recognizing Sweet’s syndrome specifically can be challenging due to its atypical clinical course, and understanding the relationship between Sweet’s syndrome and pathergy can offer an important diagnostic clue. We present a case of Sweet’s syndrome presenting with upper airway obstruction and pathergy. To our knowledge, this is the first documented case of Sweet’s syndrome presenting with oral pathergy.
Sweet’s syndrome, or acute febrile neutrophilic dermatosis, is a rare, poorly understood systemic inflammatory disorder. Originally described in 1964 by Dr. Robert Sweet, this syndrome presents with abrupt onset of erythematous or hemorrhagic rash with skin biopsy showing neutrophilic invasion of the dermis without leukocytoclastic vasculitis [
Major criteria | Minor criteria |
---|---|
Abrupt onset of characteristic rash (papules and nodules) | Fever > 38 degrees Celsius |
Biopsy proven dermal infiltration of neutrophilswithout vasculitis | Association with malignancy or inflammatory syndrome |
Excellent response to glucocorticoids | |
Elevated inflammatory markers on presentation (3 of 4) ・ ESR > 20 mm/h ・ Positive C-reactive protein ・ Leukocyte count > 10,000/ul ・ Over 70% neutrophils |
Source: Malone et al., Arch Dermatol. 2002; 138(3): 345-349. doi:10.1001/archderm.138.3.345.
A 68 years old Asian female with a past medical history of transfusion dependent myelodysplastic syndrome (MDS) presented with airway obstruction from acute oropharyngeal swelling. The patient was emergently nasally intubated for airway protection and was therefore unable to give a history, but per her family she experienced 5 days of fever, oral pain, and facial edema prior to admission. She was febrile to 39.1 degrees Celsius, tachycardic to 147 beats per minute, and mildly hypertensive to 144/87 mmHg. Physical exam showed a diffusely edematous oropharynx and tongue with scant hemorrhagic bullae. The remainder of her physical exam was normal. Her CBC, BMP and inflammatory markers are shown on
CT soft tissue neck revealed a 2.7 × 1.4 × 3.8 cm mass along the R palate and R neck. Vancomycin, clindamycin, and meropenem were started empirically, along with IV dexamethasone 4 mg q6h. Biopsy of the oral process showed inflammation with necrosis and hemorrhage, but no neoplasia. Blood and tissue cultures were negative. An autoimmune workup was performed and was negative for lupus, mixed connective tissue disease, rheumatoid arthritis, Sjogrens, vasculitis, or hereditary angioedema [
The patient’s swelling & fever improved and she was extubated hospital day four, and steroids were tapered off. Two days later, she developed recurrent fevers, oral pain and swelling. Repeat blood cultures were negative, and buccal biopsy staining and culture showed no bacteria, AFB, fungus, or neoplasm. Microscopy of the buccal mass tissue was again consistent only with chronic and acute hemorrhage with scattered mast cells and neutrophils. The return of fever after discontinuation of glucocorticoids, superimposed on the continuation of antibiotics is shown on
Three days after the removal of glucocorticoids, the patient developed painful erythematous plaques on her trunk and also induration of the right forearm concerning for necrotizing fasciitis [
This patient originally presented with upper airway obstruction, which was later found to be the initial symptom
Complete blood count | |
---|---|
WBC | 4.1 × 103 |
RBC | 3.5 × 103 |
HGB | 9.8 |
HCT | 28.9 |
Platelets | <10 × 103 |
Segs (%) | 53% |
Blasts (%) | 7% |
Basic metabolic panel | |
Glucose | 178 mg/dl |
BUN | 13 mg/dl |
Creatinine | 0.3 mg/dl |
Sodium | 138 mEq/L |
Potassium | 4.1 mEq/L |
Chloride | 106 mEq/L |
CO2 | 21 mEq/L |
Sedimentation rate | 108 mm/hr |
---|---|
C reactive protein | 26.8 mg/dl |
ANA screen | Negative |
DS DNA screen | Negative |
ENA Smith | Negative |
ENA RNP | Negative |
Rheumatoid factor | Negative |
SS A antibody | Negative |
SS B antibody | Negative |
C-ANCA | <1:20 |
P-ANCA | <1:20 |
C1 esterase inhibitor | 39 (within normal limits) |
C3 complement | 103 (within normal limits) |
C4 complement | 25 (within normal limits) |
of Sweet’s syndrome. At the time of presentation, there was no history of oral trauma, which made identifying the oral lesion a possible pathergy response diagnostically impossible. It was only after the patient developed pathergy to her surgical incision that the diagnosis became clear. With subsequent skin biopsy positive for neutrophilic infiltrate without leukocytoclastic vasculitis, characteristic truncal rash, hematologic malignancy, fever, and resolution of symptoms with glucocorticoids, she fit both major and three out of four minor criteria for Sweet’s.
Sweet’s syndrome can present with has a variety of clinical phenomena that are not a part of its diagnostic criteria of inflammation and a papular/nodular rash. Solid organ involvement has been seen including encephalitis [
The pathergy response can be seen in a number of disorders, most notably Behcet’s syndrome, where an inflammatory response to pinprick injury is part of the diagnostic criteria [
We chose to treat the patient with prednisone 60 mg/d. Prednisone 0.5 - 1 mg/kg/d is the standard dosing protocol for Sweet’s syndrome to be continued for at least 4 - 6 weeks [
Making an initial diagnosis of Sweet’s syndrome can be difficult due to its somewhat ambiguous presentation of systemic inflammation and rash, as well as the need for a skin biopsy. Without a very high index of suspicion, diagnosis can often be delayed resulting in patient morbidity. It is important for clinicians to recognize pathergy as a sign of Sweet’s syndrome, and pursue a skin biopsy if new pathergy is observed in a patient with unexplained systemic inflammation. This is the first documented case of Sweet’s syndrome presenting with oral pathergy.
Special thanks to Dr. John Snellings.
David Klimpl,Thomas Manser,Mark Flemmer, (2016) Oral Pathergy in Sweet’s Syndrome Following Food Bolus Injury. Case Reports in Clinical Medicine,05,134-139. doi: 10.4236/crcm.2016.54025