Transcript A Case of Unusual Rash Associated with Cutaneous Trauma

The Sweet Spot: A Case of Unusual Rash Associated
with Cutaneous Trauma
LT Laura Gilbert, MD, Associate
CPT Nate Copeland, MD, Associate
Catherine F. Decker, MD, Fellow
Navy ACP Conference
Walter Reed National Military Medical Center
October 17, 2014
Case Presentation
• 68yF presents with diffuse erythematous,
non-pruritic and painful lesions involving face,
legs, chest, and back
• Endorses low grade fever
• Placed on TMP-SMX post I/D of a left axillary
MRSA abscess nine days PTA
Past Medical/Surgical History
• GERD, hyperlipidemia, overactive bladder
• Hysterectomy
• Left total knee arthroplasty Jan 2014
– Complicated by post-op MRSA joint infection in
Mar 2014
• Left axillary MRSA abscess s/p I&D Aug 2014
– Started on TMP-SMX 9 days prior to presentation
Social History
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Non-smoker, rare alcohol, no IV drug use
Originally from Thailand
No recent travel, sick contacts or tick exposure
Swimming in pool 4 days prior to presentation
Dog at home, no new pet or animal exposure
Medications
• Outpatient Medications:
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Atorvastatin
Aspirin
Esomeprazole
Conjugated Estrogen
Solifenacin Succinate
TMP-SMX
• Allergies:
– Shellfish
Physical Exam
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Vital signs: afebrile
HEENT: no conjunctival injection, oral ulcers
Extremities: no edema
Skin:
Laboratory Data
• Complete blood count:
– WBC 9.9K, Hgb 11.9 g/dL, Plt 36K (repeat - 22K)
• Serum chemistry: normal
• Urinalysis: normal
• ESR: 43 mm/hr
• CRP: 9.475 mg/dL
• Skin biopsy:
Neutrophilic Dermatosis: Differential Diagnosis
• Infections
– Bacterial, Fungal, Mycobacterial
• Inflammatory diseases
– Vasculitis: Polyarteritis nodosa
– Erythema nodosum, Pyoderma gangrenosum
– Acne fulminans, Psoriasis
– Cutaneous Sarcoidosis
– Behçet's disease
– Sweet Syndrome
Sweet Syndrome
• First described in 1964
– “Acute febrile
neutrophilic dermatosis”
– Initially called GommButton disease
– First reported druginduced case in 1986
• TMP-SMX
Characteristics
• Fever with characteristic rash and pathology
– Neutrophilic dermatosis without vasculitis
– Rash: painful, raised erythematous papules
• 3 Major classifications
– Classical
– Malignancy-associated
– Drug-induced
Classification
• Classical
– Infections, Inflammatory Bowel Disease, Pregnancy
• Malignancy-associated
• Drug-induced
– Granulocyte-colony stimulating factor, antibiotics,
chemotherapy
Clinical Features
Cohen PR. Sweet’s syndrome – a comprehensive review of an acute febrile neutrophilic dermatosis.
Orphanet J Rare Dis. 2007 Jul; 2:34.
Diagnostic Criteria: Drug-induced
• Sudden onset of painful, erythematous nodules
• Neutrophilic infiltration of dermis without vasculitis
• Fever > 38 C
• Relationship between drug ingestion and symptoms
• Improvement in symptoms after discontinuation of
medication or treatment with steroids
Pathophysiology
• Not well understood
• Hypersensitivity reaction
– Immune reaction to antigen: activation cytokines
and PMNs
• Cytokine dysregulation
• Genetic susceptibility
– HLA B54 linked to Sweet in Japanese women
Management
• Removal of inciting drug if applicable
• Treatment of underlying condition
• First line agents:
– oral steroids or potassium iodide
• Second line agents:
– Dapsone or Colchicine
Hospital Course
• TMP-SMX and Aspirin were discontinued
• Dermatology recommendation: no oral steroids
• Daily CBC
– Platelet count recovered to 118K by HD 3
• Decrease in erythema
• Recommended age-appropriate cancer screening
• TMP-SMX was listed as an allergy
Sweet Syndrome and Pathergy
• Definition: hyper-reactivity of the skin in
response to cutaneous trauma
• Rare association with Sweet syndrome
– Case reports associated with burns, radiation, biopsy
site, bites, surgical scars
• First documented case of Sweet syndrome
associated with non-acute trauma?
Conclusion
• Sweet syndrome mimics a variety of diseases
characterized by rash and a fever
• Association with malignancy and drugs
• Biopsy is essential for diagnosis
• Treatment with oral steroids is gold standard
• Pathergy is rare clinical manifestation of Sweet
syndrome associated with cutaneous trauma
References
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Awan F, Hamadani M, Devine S. Paraneoplastic Sweet’s Syndrome and the pathergy phenomenon. 2007; 86:613-614.
Bi XL, Gu J, Yan M, Gao CF. A case of Sweet's syndrome with slack skin and pathergy phenomenon. Int J Dermatol. 2008 Aug;
47(8):842-4.
Callen JP. Neutrophilic Dermatosis. Derm Clinics. 2002 Jul; 20:3.
Cohen PR. Sweet’s syndrome – a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J Rare Dis.
2007 Jul; 2:34.
De Moya MA, Wong JT, Kroshinsky D, Robbins GK, Shenoy-Bhangle AS, Gimbel DC. Case records of the Massachusetts
General Hospital. Case 28-2012. A 30-year-old woman with shock and abdominal-wall necrosis after cesarean section. N
Engl J Med. 2012 Sep 13; 367(11):1046-57.
Minocha R, Sebaratnam D, Choi J. Sweet’s Syndrome following surgery: cutaneous trauma as a possible aetiological co-factor
in neutrophilic dermatosis. Australias J Derm. Online publication: March 11, 2014.
O’Halloran E, Stewart N, Vetrichevvel TP, Rea S, Wood F. Sweet’s syndrome mimicking alkali burn: a clinical conundrum. J
Plast Reconstr Aesthet Surg. 2013 Jun; 66(6):867-869.
Ramos I, Wiering C, Tebcherani A, Sanchez A. Sweet’s syndrome on surgical scar. An Bras Dermatol. 2006; 81(5):S324-6.
Sweet RD. Br J Dermatol. 1964; 76:349-356.
Varol A, Seifert O, Anderson C. The skin pathergy test: innately useful? Arch Dermatol Res. 2010; 302:155-168.
Walker DC, Cohen PR. Trimethoprim-sulfamethoxazole-associated acute febrile neutrophilic dermatosis: Case report and a
review of drug-induced Sweet’s Syndrome. J Am Acad Derm. 1996; 34:918-923.
Thank you!