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40-year-old woman • fever • rash • arthralgia • Dx?
THE CASE
A 40-year-old woman with no significant medical history sought care at the emergency department for a fever, rash, and arthralgia. On admission, she had worsening bilateral ankle pain and was having difficulty walking. During the previous 3 months, she’d had 3 episodes of tonsillitis, all of which were presumed to be caused by Streptococcus, although no swabs were obtained. Her primary care physician treated her with antibiotics each time: 1 round of amoxicillin 500 mg twice daily for 10 days and 2 rounds of amoxicillin/clavulanate 875 mg twice daily for 7 to 10 days. During the previous month, she’d experienced intermittent fevers ranging from 100.2 °F to 100.8
The patient said that 2 weeks prior to her admission to the hospital, she’d developed a rash on her right arm, which was papular, nondraining, nonpruritic, and not painful (FIGURE 1). Six days later, the rash spread to her left arm, chest, and back, with a few lesions on her legs (FIGURE 2). A few days later, she developed arthralgias in her hips, knees, and ankles. These were associated with the appearance of large, flat, erythematous lesions on her anterior lower extremities (FIGURE 2). About 5 days before she was admitted to our hospital, the patient was seen at another hospital and treated for possible cellulitis with cephalexin (500 mg 4 times daily for 5-7 days), but her symptoms persisted.
At this point, she sought care at our hospital for her worsening lower extremity arthralgia, difficulty walking, and the persistent rash. An initial lab report showed a white blood cell (WBC) count of 12.6 × 103/µL (normal range, 4.0-10.0 × 103/µL) with an absolute neutrophil count of 9.7 × 103/µL (normal, 1.7-7.0 × 103/µL). Her C-reactive protein (CRP) level was elevated (194.7 mg/L; normal, 0.0-5.0 mg/L), as was her erythrocyte sedimentation rate (ESR) (102.0 mm/h; normal, 0.0-20.0 mm/h). A rapid pharyngeal strep test was negative. Her anti-streptolysin O (ASO) titer was elevated (2092.0 IU/mL; normal, < 250.0 IU/mL), and her rheumatic factor was mildly elevated (19.0 IU/mL; normal, 0.0-14.0 IU/mL). An antinuclear antibody panel was positive at 1:80. Further testing was performed, and the patient was found to be negative for Sjögren syndrome A, Sjögren syndrome B, anti-Smith, scleroderma-70, double-stranded DNA, and chromatin AB—making an autoimmune disease unlikely.
THE DIAGNOSIS
The patient met the American Heart Association’s revised Jones criteria for the diagnosis of rheumatic fever: She had a positive ASO titer; polyarthritis and subcutaneous nodules (2 major criteria); and ESR > 60 mm/h and CRP > 3 mg/L (1 minor criterion).1 She started taking naproxen 500 mg twice per day and was given a penicillin G 1.5-million-unit injection. A transthoracic echocardiogram also was performed during her admission to rule out endocarditis; no abnormalities were found.
A few days after starting treatment for rheumatic fever, the patient’s WBC count returned to within normal limits and her joint swelling and pain improved; however, her rash did not go away, leading us to wonder if there was a second disease at work. Dermatology was consulted, and a punch biopsy was obtained. The results showed acute febrile neutrophilic dermatosis, or Sweet syndrome.
DISCUSSION
Sweet syndrome is considered rare, and incidence numbers are elusive.2 It has a worldwide distribution and no racial bias.3 Sweet syndrome usually occurs in women ages 30 to 50 years, although it may also occur in younger adults and children.
Three subtypes have been defined based on etiology: (1) classical (or idiopathic) Sweet syndrome; (2) malignancy-associated Sweet syndrome, which is most often related to acute myelogenous leukemia; and (3) drug-induced Sweet syndrome, which is usually associated with granulocyte colony–stimulating factor treatment.4 Our patient had the most common subtype: classical Sweet syndrome.
Continue to: What you'll see
What you’ll see.
Corticosteroid therapy is the gold standard for treatment of classical Sweet syndrome.Dosing usually starts with prednisone 1 mg/kg/d, which can be tapered to 10 mg/d within 4 to 6 weeks.5 If steroid treatment is contraindicated in the patient, alternative treatments are colchicine 0.5 mg 3 times daily for 10 to 21 days or enteric-coated potassium iodide 300 mg 3 times daily until the rash subsides.5 Without treatment, symptoms may resolve within weeks to months; with treatment, the rash usually resolves within 2 to 5 days. Some resistant forms may require 2 to 3 months of treatment.
There is a risk of recurrence in approximately one-third of patients after successful treatment of classical Sweet syndrome.5 Recurrence can be caused by another inciting factor (ie, irritable bowel disease, upper respiratory tract infection, malignancy, or a new medication), making a new investigation necessary. However, treatment would entail the same medications.5
The patient was placed on penicillin V 250 mg twice daily for 5 years due to the significant risk of carditis in the setting of rheumatic fever. She started an oral steroid regimen of a prednisone weekly taper, starting with 60 mg/d, for 4 to 6 weeks. Her papular rash improved soon after initiation of steroid therapy.
THE TAKEAWAY
On presentation, this patient’s symptoms met the Jones criteria for rheumatic fever, but she did not respond to treatment. This led us to revisit her case, order additional tests, and identify a second diagnosis—Sweet syndrome—that responded positively to treatment. This case is a reminder that sometimes the signs and symptoms we are looking at are the result of 2 underlying illnesses, with 1 possibly triggering the other. That was likely what occurred in this case.
Farah Leclercq, DO, Department of Family Medicine, University of Florida, 12041 Southwest 1 Lane, Gainesville, FL 32607; [email protected]
1. Gewitz MH, Baltimore SR, Tani LY, et al. Revision of the Jones Criteria for the diagnosis of acute rheumatic fever in the era of doppler echocardiography: a scientific statement from the American Heart Association. Circulation. 2015;131:1806-1818. doi: 10.1161/CIR.0000000000000205
2. Joshi TP, Friske SK, Hsiou DA, Duvic M. New practical aspects of Sweet syndrome. Am J Clin Dermatol. 2022;23:301-318. doi: 10.1007/s40257-022-00673-4
3. Cohen PR, Kurzrock R. Sweets syndrome revisited: a review of disease concepts. Int J Dermatol. 2003;42:761-778. doi: 10.1046/j.1365-4362.2003.01891.x
4. Merola JF. Sweet syndrome (acute febrile neutrophilic dermatosis): pathogenesis, clinical manifestations, and diagnosis. UpToDate. August 9, 2020. Accessed October 27, 2022. www.uptodate.com/contents/sweet-syndrome-acute-febrile-neutrophilic-dermatosis-pathogenesis-clinical-manifestations-and-diagnosis
5. Cohen PR. Sweets syndrome—a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J Rare Dis. 2007;2:34. doi: 10.1186/1750-1172-2-34
THE CASE
A 40-year-old woman with no significant medical history sought care at the emergency department for a fever, rash, and arthralgia. On admission, she had worsening bilateral ankle pain and was having difficulty walking. During the previous 3 months, she’d had 3 episodes of tonsillitis, all of which were presumed to be caused by Streptococcus, although no swabs were obtained. Her primary care physician treated her with antibiotics each time: 1 round of amoxicillin 500 mg twice daily for 10 days and 2 rounds of amoxicillin/clavulanate 875 mg twice daily for 7 to 10 days. During the previous month, she’d experienced intermittent fevers ranging from 100.2 °F to 100.8
The patient said that 2 weeks prior to her admission to the hospital, she’d developed a rash on her right arm, which was papular, nondraining, nonpruritic, and not painful (FIGURE 1). Six days later, the rash spread to her left arm, chest, and back, with a few lesions on her legs (FIGURE 2). A few days later, she developed arthralgias in her hips, knees, and ankles. These were associated with the appearance of large, flat, erythematous lesions on her anterior lower extremities (FIGURE 2). About 5 days before she was admitted to our hospital, the patient was seen at another hospital and treated for possible cellulitis with cephalexin (500 mg 4 times daily for 5-7 days), but her symptoms persisted.
At this point, she sought care at our hospital for her worsening lower extremity arthralgia, difficulty walking, and the persistent rash. An initial lab report showed a white blood cell (WBC) count of 12.6 × 103/µL (normal range, 4.0-10.0 × 103/µL) with an absolute neutrophil count of 9.7 × 103/µL (normal, 1.7-7.0 × 103/µL). Her C-reactive protein (CRP) level was elevated (194.7 mg/L; normal, 0.0-5.0 mg/L), as was her erythrocyte sedimentation rate (ESR) (102.0 mm/h; normal, 0.0-20.0 mm/h). A rapid pharyngeal strep test was negative. Her anti-streptolysin O (ASO) titer was elevated (2092.0 IU/mL; normal, < 250.0 IU/mL), and her rheumatic factor was mildly elevated (19.0 IU/mL; normal, 0.0-14.0 IU/mL). An antinuclear antibody panel was positive at 1:80. Further testing was performed, and the patient was found to be negative for Sjögren syndrome A, Sjögren syndrome B, anti-Smith, scleroderma-70, double-stranded DNA, and chromatin AB—making an autoimmune disease unlikely.
THE DIAGNOSIS
The patient met the American Heart Association’s revised Jones criteria for the diagnosis of rheumatic fever: She had a positive ASO titer; polyarthritis and subcutaneous nodules (2 major criteria); and ESR > 60 mm/h and CRP > 3 mg/L (1 minor criterion).1 She started taking naproxen 500 mg twice per day and was given a penicillin G 1.5-million-unit injection. A transthoracic echocardiogram also was performed during her admission to rule out endocarditis; no abnormalities were found.
A few days after starting treatment for rheumatic fever, the patient’s WBC count returned to within normal limits and her joint swelling and pain improved; however, her rash did not go away, leading us to wonder if there was a second disease at work. Dermatology was consulted, and a punch biopsy was obtained. The results showed acute febrile neutrophilic dermatosis, or Sweet syndrome.
DISCUSSION
Sweet syndrome is considered rare, and incidence numbers are elusive.2 It has a worldwide distribution and no racial bias.3 Sweet syndrome usually occurs in women ages 30 to 50 years, although it may also occur in younger adults and children.
Three subtypes have been defined based on etiology: (1) classical (or idiopathic) Sweet syndrome; (2) malignancy-associated Sweet syndrome, which is most often related to acute myelogenous leukemia; and (3) drug-induced Sweet syndrome, which is usually associated with granulocyte colony–stimulating factor treatment.4 Our patient had the most common subtype: classical Sweet syndrome.
Continue to: What you'll see
What you’ll see.
Corticosteroid therapy is the gold standard for treatment of classical Sweet syndrome.Dosing usually starts with prednisone 1 mg/kg/d, which can be tapered to 10 mg/d within 4 to 6 weeks.5 If steroid treatment is contraindicated in the patient, alternative treatments are colchicine 0.5 mg 3 times daily for 10 to 21 days or enteric-coated potassium iodide 300 mg 3 times daily until the rash subsides.5 Without treatment, symptoms may resolve within weeks to months; with treatment, the rash usually resolves within 2 to 5 days. Some resistant forms may require 2 to 3 months of treatment.
There is a risk of recurrence in approximately one-third of patients after successful treatment of classical Sweet syndrome.5 Recurrence can be caused by another inciting factor (ie, irritable bowel disease, upper respiratory tract infection, malignancy, or a new medication), making a new investigation necessary. However, treatment would entail the same medications.5
The patient was placed on penicillin V 250 mg twice daily for 5 years due to the significant risk of carditis in the setting of rheumatic fever. She started an oral steroid regimen of a prednisone weekly taper, starting with 60 mg/d, for 4 to 6 weeks. Her papular rash improved soon after initiation of steroid therapy.
THE TAKEAWAY
On presentation, this patient’s symptoms met the Jones criteria for rheumatic fever, but she did not respond to treatment. This led us to revisit her case, order additional tests, and identify a second diagnosis—Sweet syndrome—that responded positively to treatment. This case is a reminder that sometimes the signs and symptoms we are looking at are the result of 2 underlying illnesses, with 1 possibly triggering the other. That was likely what occurred in this case.
Farah Leclercq, DO, Department of Family Medicine, University of Florida, 12041 Southwest 1 Lane, Gainesville, FL 32607; [email protected]
THE CASE
A 40-year-old woman with no significant medical history sought care at the emergency department for a fever, rash, and arthralgia. On admission, she had worsening bilateral ankle pain and was having difficulty walking. During the previous 3 months, she’d had 3 episodes of tonsillitis, all of which were presumed to be caused by Streptococcus, although no swabs were obtained. Her primary care physician treated her with antibiotics each time: 1 round of amoxicillin 500 mg twice daily for 10 days and 2 rounds of amoxicillin/clavulanate 875 mg twice daily for 7 to 10 days. During the previous month, she’d experienced intermittent fevers ranging from 100.2 °F to 100.8
The patient said that 2 weeks prior to her admission to the hospital, she’d developed a rash on her right arm, which was papular, nondraining, nonpruritic, and not painful (FIGURE 1). Six days later, the rash spread to her left arm, chest, and back, with a few lesions on her legs (FIGURE 2). A few days later, she developed arthralgias in her hips, knees, and ankles. These were associated with the appearance of large, flat, erythematous lesions on her anterior lower extremities (FIGURE 2). About 5 days before she was admitted to our hospital, the patient was seen at another hospital and treated for possible cellulitis with cephalexin (500 mg 4 times daily for 5-7 days), but her symptoms persisted.
At this point, she sought care at our hospital for her worsening lower extremity arthralgia, difficulty walking, and the persistent rash. An initial lab report showed a white blood cell (WBC) count of 12.6 × 103/µL (normal range, 4.0-10.0 × 103/µL) with an absolute neutrophil count of 9.7 × 103/µL (normal, 1.7-7.0 × 103/µL). Her C-reactive protein (CRP) level was elevated (194.7 mg/L; normal, 0.0-5.0 mg/L), as was her erythrocyte sedimentation rate (ESR) (102.0 mm/h; normal, 0.0-20.0 mm/h). A rapid pharyngeal strep test was negative. Her anti-streptolysin O (ASO) titer was elevated (2092.0 IU/mL; normal, < 250.0 IU/mL), and her rheumatic factor was mildly elevated (19.0 IU/mL; normal, 0.0-14.0 IU/mL). An antinuclear antibody panel was positive at 1:80. Further testing was performed, and the patient was found to be negative for Sjögren syndrome A, Sjögren syndrome B, anti-Smith, scleroderma-70, double-stranded DNA, and chromatin AB—making an autoimmune disease unlikely.
THE DIAGNOSIS
The patient met the American Heart Association’s revised Jones criteria for the diagnosis of rheumatic fever: She had a positive ASO titer; polyarthritis and subcutaneous nodules (2 major criteria); and ESR > 60 mm/h and CRP > 3 mg/L (1 minor criterion).1 She started taking naproxen 500 mg twice per day and was given a penicillin G 1.5-million-unit injection. A transthoracic echocardiogram also was performed during her admission to rule out endocarditis; no abnormalities were found.
A few days after starting treatment for rheumatic fever, the patient’s WBC count returned to within normal limits and her joint swelling and pain improved; however, her rash did not go away, leading us to wonder if there was a second disease at work. Dermatology was consulted, and a punch biopsy was obtained. The results showed acute febrile neutrophilic dermatosis, or Sweet syndrome.
DISCUSSION
Sweet syndrome is considered rare, and incidence numbers are elusive.2 It has a worldwide distribution and no racial bias.3 Sweet syndrome usually occurs in women ages 30 to 50 years, although it may also occur in younger adults and children.
Three subtypes have been defined based on etiology: (1) classical (or idiopathic) Sweet syndrome; (2) malignancy-associated Sweet syndrome, which is most often related to acute myelogenous leukemia; and (3) drug-induced Sweet syndrome, which is usually associated with granulocyte colony–stimulating factor treatment.4 Our patient had the most common subtype: classical Sweet syndrome.
Continue to: What you'll see
What you’ll see.
Corticosteroid therapy is the gold standard for treatment of classical Sweet syndrome.Dosing usually starts with prednisone 1 mg/kg/d, which can be tapered to 10 mg/d within 4 to 6 weeks.5 If steroid treatment is contraindicated in the patient, alternative treatments are colchicine 0.5 mg 3 times daily for 10 to 21 days or enteric-coated potassium iodide 300 mg 3 times daily until the rash subsides.5 Without treatment, symptoms may resolve within weeks to months; with treatment, the rash usually resolves within 2 to 5 days. Some resistant forms may require 2 to 3 months of treatment.
There is a risk of recurrence in approximately one-third of patients after successful treatment of classical Sweet syndrome.5 Recurrence can be caused by another inciting factor (ie, irritable bowel disease, upper respiratory tract infection, malignancy, or a new medication), making a new investigation necessary. However, treatment would entail the same medications.5
The patient was placed on penicillin V 250 mg twice daily for 5 years due to the significant risk of carditis in the setting of rheumatic fever. She started an oral steroid regimen of a prednisone weekly taper, starting with 60 mg/d, for 4 to 6 weeks. Her papular rash improved soon after initiation of steroid therapy.
THE TAKEAWAY
On presentation, this patient’s symptoms met the Jones criteria for rheumatic fever, but she did not respond to treatment. This led us to revisit her case, order additional tests, and identify a second diagnosis—Sweet syndrome—that responded positively to treatment. This case is a reminder that sometimes the signs and symptoms we are looking at are the result of 2 underlying illnesses, with 1 possibly triggering the other. That was likely what occurred in this case.
Farah Leclercq, DO, Department of Family Medicine, University of Florida, 12041 Southwest 1 Lane, Gainesville, FL 32607; [email protected]
1. Gewitz MH, Baltimore SR, Tani LY, et al. Revision of the Jones Criteria for the diagnosis of acute rheumatic fever in the era of doppler echocardiography: a scientific statement from the American Heart Association. Circulation. 2015;131:1806-1818. doi: 10.1161/CIR.0000000000000205
2. Joshi TP, Friske SK, Hsiou DA, Duvic M. New practical aspects of Sweet syndrome. Am J Clin Dermatol. 2022;23:301-318. doi: 10.1007/s40257-022-00673-4
3. Cohen PR, Kurzrock R. Sweets syndrome revisited: a review of disease concepts. Int J Dermatol. 2003;42:761-778. doi: 10.1046/j.1365-4362.2003.01891.x
4. Merola JF. Sweet syndrome (acute febrile neutrophilic dermatosis): pathogenesis, clinical manifestations, and diagnosis. UpToDate. August 9, 2020. Accessed October 27, 2022. www.uptodate.com/contents/sweet-syndrome-acute-febrile-neutrophilic-dermatosis-pathogenesis-clinical-manifestations-and-diagnosis
5. Cohen PR. Sweets syndrome—a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J Rare Dis. 2007;2:34. doi: 10.1186/1750-1172-2-34
1. Gewitz MH, Baltimore SR, Tani LY, et al. Revision of the Jones Criteria for the diagnosis of acute rheumatic fever in the era of doppler echocardiography: a scientific statement from the American Heart Association. Circulation. 2015;131:1806-1818. doi: 10.1161/CIR.0000000000000205
2. Joshi TP, Friske SK, Hsiou DA, Duvic M. New practical aspects of Sweet syndrome. Am J Clin Dermatol. 2022;23:301-318. doi: 10.1007/s40257-022-00673-4
3. Cohen PR, Kurzrock R. Sweets syndrome revisited: a review of disease concepts. Int J Dermatol. 2003;42:761-778. doi: 10.1046/j.1365-4362.2003.01891.x
4. Merola JF. Sweet syndrome (acute febrile neutrophilic dermatosis): pathogenesis, clinical manifestations, and diagnosis. UpToDate. August 9, 2020. Accessed October 27, 2022. www.uptodate.com/contents/sweet-syndrome-acute-febrile-neutrophilic-dermatosis-pathogenesis-clinical-manifestations-and-diagnosis
5. Cohen PR. Sweets syndrome—a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J Rare Dis. 2007;2:34. doi: 10.1186/1750-1172-2-34
