Abrupt Onset of a Tender Rash on the Upper and Lower Extremities
Am Fam Physician. 2018 Jan 15;97(2):129-134.
A 54-year-old woman presented with painful lesions on her upper and lower extremities that developed a few weeks earlier. The rash did not improve after two courses of antibiotics. She had a fever and severe generalized pain. Her medical history was unremarkable.
Physical examination revealed an erythematous rash with vesicles and papules (Figure 1). Her temperature was 101°F (38°C). She was admitted for intravenous antibiotic treatment, pain control, and infectious disease consultation. Initial laboratory results showed anemia, thrombocytopenia, elevated erythrocyte sedimentation rate, and elevated C-reactive protein level.
Based on the patient's history, physical examination, and laboratory findings, which one of the following is the most likely diagnosis?
A. Contact dermatitis.
B. Drug eruption rash.
C. Erythema multiforme.
D. Erythema nodosum.
E. Sweet syndrome.
The answer is E: Sweet syndrome. Sweet syndrome, or acute febrile neutrophilic dermatitis, is characterized by painful papules, nodules, or plaques caused by an accumulation of neutrophils in the dermis.1 Multiple tender, erythematous lesions develop on the skin or mucosa, most commonly on the face, neck, and upper and lower extremities.1 There are three forms of Sweet syndrome: classic, malignancy associated, and drug induced.2,3 Diagnosis includes major and minor criteria. Major criteria are the rapid onset of painful erythematous plaques and nodules, and typical histopathology of dense neutrophil infiltration without leukocytoclastic vasculitis.4 Minor criteria include fever higher than 100.4°F (38°C), erythrocyte sedimentation rate greater than 20 mm per hour, white blood cell count greater than 8,000 per mm3 (8 × 109 per L), neutrophils greater than 70%, and an elevated C-reactive protein level.4
Sweet syndrome is often diagnosed in patients who have a history of upper respiratory tract
Referencesshow all references
1. Wolff K, Johnson RA, Suurmond D. Fitzpatrick's Color Atlas and Synopsis of Clinical Dermatology. 5th ed. New York, NY: McGraw-Hill; 2005....
2. Cohen PR. Sweet's syndrome—a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J Rare Dis. 2007;2:34.
3. Soto R, Levy Y, Krause JR. Sweet syndrome and its association with hematopoietic neoplasms. Proc (Bayl Univ Med Cent). 2015;28(1):62–64.
4. Paydas S. Sweet's syndrome: a revisit for hematologists and oncologists. Crit Rev Oncol Hematol. 2013;86(1):85–95.
This series is coordinated by John E. Delzell Jr., MD, MSPH, Assistant Medical Editor.
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