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Heart failure in acute febrile neutrophilic dermatosis
CASE REPORT
Case report
Heart failure in acute febrile neutrophilic dermatosis
Marc Dorenkamp, Ulf Weikert, Rudolf Meyer, Peter L Schwimmbeck, Andreas J Morguet A 46-year-old, previously healthy, man presented in January, 2003, with a 1-week history of a painful left knee effusion. His general practitioner aspirated the joint, analysed the synovial fluid, and excluded septic and crystal-induced arthritis. The patient suddenly developed a high fever and erythematous, oedematous, plaques on the limbs. He was referred to the department of dermatology at our institute. A white blood count showed neutrophilia (neutrophils 77·5%, lymphocytes 11·2%, and monocytes 9·7%). C-reactive protein was 143 mg/L. Doctors did blood tests for antibodies to hepatitis viruses, and Yersinia, Chlamydia, Mycoplasma and Toxoplasma, and screened for autoimmune diseases. Only the serology against Yersinia enterocolitica was positive and increased antibodies (IgA 12·0 U/mL, IgG 30·2 U/mL) were confirmed by western blot. A skin nodule biopsy showed diffuse neutrophilic infiltration of the dermis. There was no evidence of underlying malignancy. The dermatologists diagnosed acute febrile neutrophilic dermatosis (Sweet’s syndrome), preceded by a recent Yersinia infection and started doxycycline, 100 mg daily and prednisolone, 30 mg daily. The fever dropped and the plaques started to fade. During the second week in hospital, the patient developed a new diastolic heart murmur. We did transthoracic and transoesophageal echocardiography and found a dilated left ventricle (left ventricular end-diastolic diameter [LVEDD], 61 mm) with slightly impaired systolic function and a mildly incompetent bicuspid aortic valve without vegetations. Serial blood cultures were negative. By the time of discharge 2 weeks later, the patient’s skin symptoms had almost resolved. 2 weeks after discharge, during scheduled follow-up in the department of cardiology, the patient complained of exertional dyspnoea and dizziness. He had de Musset’s sign and a high pulse pressure (160/55 mm Hg). Repeat echocardiography showed increased left ventricular dilatation (LVEDD, 75 mm) and severe aortic regurgitation. There were no vegetations. Cardiac catheterisation showed grade III aortic insufficiency and excluded coronary artery disease. Transfemoral right ventricular endomyocardial biopsy showed marked intersitital neutrophilic infiltration and scattered T lymphocytes and mast cells (figure, A). The patient had prompt aortic valve replacement with a 27-mm St Jude
A
50 m
Endomyocardial biopsy sample (A) and excised aortic value cusp (B) Showing interstitial neutrophils (A, arrow) and focal degeneration and subendocardial macrophage activation (B, arrow) (haematoxilin-eosin ⫻20).
Medical prosthesis. The aortic valve had gross perforations, and focal fibrosal and mucoid degeneration and subendocardial macrophage infiltration on histological examination (figure, B). No micro-organisms or microthrombi characteristic of endocarditis were found in the surgical specimen (gram stains, acid-fast satins, and Grocott stains were negative). Postoperatively, the patient’s left ventricular function improved under tapered steroid therapy. When last seen in August, 2003, he had no complaints and his LVEDD was 62 mm. Sweet’s syndrome is characterised by the sudden onset of fever, neutrophilia, and rash1 (erythematous, welldemarcated papules and plaques with dense white cell infiltrates).2 Associated conditions include malignancies, infections, drug reactions, and autoimmune diseases.3 Cardiomegaly and acute valvulitis are also complications of Sweet’s syndrome.4,5 In addition to the usual extracutaneous manifestations, cardiac involvement can cause problems in patients with Sweet’s syndrome. We thank Dr Chalid Assaf for diagnosing Sweet’s syndrome and referring this patient to us.
References 1
Lancet 2003; 362: 1374 Department of Cardiology and Pneumology (M Dorenkamp MD, U Weikert MD, P L Schwimmbeck MD, A J Morguet MD), Benjamin Franklin University Hospital, 12200 Berlin, and Department of Pathology (R Meyer MD), German Heart Institute, 13353 Berlin, Germany Correspondence to: Dr Andreas J Morguet (e-mail: [email protected])
1374
B
2
3 4 5
Sweet RD. An acute febrile neutrophilic dermatosis. Br J Dermatol 1964; 139: 349–56. Delabie J, De Wolf-Peeters C, Mooren M, Karien K, Roskams T, Desmet V. Histiocytes in Sweet’s syndrome. Br J Dermatol 1991; 124: 348–53. Callen JP. Neutrophilic dermatoses. Dermatol Clin 2002; 20: 409–19. Shimizu K. Neutrophilic infiltration of myocardium in a patient with myelodysplastic syndrome. Am J Hematol 1998; 58: 337–38. Hayashi I, Hosoda Y, Kawasaki S, Yamamoto T, Dohi S, Kawai S. Aortic and mitral valve replacement in a patient with acute neutrophilic dermatosis (Sweet’s syndrome): report of a case. Surg Today 2001; 31: 810–13.
THE LANCET • Vol 362 • October 25, 2003 • www.thelancet.com
For personal use. Only reproduce with permission from The Lancet publishing Group.
Case report
Heart failure in acute febrile neutrophilic dermatosis
Marc Dorenkamp, Ulf Weikert, Rudolf Meyer, Peter L Schwimmbeck, Andreas J Morguet A 46-year-old, previously healthy, man presented in January, 2003, with a 1-week history of a painful left knee effusion. His general practitioner aspirated the joint, analysed the synovial fluid, and excluded septic and crystal-induced arthritis. The patient suddenly developed a high fever and erythematous, oedematous, plaques on the limbs. He was referred to the department of dermatology at our institute. A white blood count showed neutrophilia (neutrophils 77·5%, lymphocytes 11·2%, and monocytes 9·7%). C-reactive protein was 143 mg/L. Doctors did blood tests for antibodies to hepatitis viruses, and Yersinia, Chlamydia, Mycoplasma and Toxoplasma, and screened for autoimmune diseases. Only the serology against Yersinia enterocolitica was positive and increased antibodies (IgA 12·0 U/mL, IgG 30·2 U/mL) were confirmed by western blot. A skin nodule biopsy showed diffuse neutrophilic infiltration of the dermis. There was no evidence of underlying malignancy. The dermatologists diagnosed acute febrile neutrophilic dermatosis (Sweet’s syndrome), preceded by a recent Yersinia infection and started doxycycline, 100 mg daily and prednisolone, 30 mg daily. The fever dropped and the plaques started to fade. During the second week in hospital, the patient developed a new diastolic heart murmur. We did transthoracic and transoesophageal echocardiography and found a dilated left ventricle (left ventricular end-diastolic diameter [LVEDD], 61 mm) with slightly impaired systolic function and a mildly incompetent bicuspid aortic valve without vegetations. Serial blood cultures were negative. By the time of discharge 2 weeks later, the patient’s skin symptoms had almost resolved. 2 weeks after discharge, during scheduled follow-up in the department of cardiology, the patient complained of exertional dyspnoea and dizziness. He had de Musset’s sign and a high pulse pressure (160/55 mm Hg). Repeat echocardiography showed increased left ventricular dilatation (LVEDD, 75 mm) and severe aortic regurgitation. There were no vegetations. Cardiac catheterisation showed grade III aortic insufficiency and excluded coronary artery disease. Transfemoral right ventricular endomyocardial biopsy showed marked intersitital neutrophilic infiltration and scattered T lymphocytes and mast cells (figure, A). The patient had prompt aortic valve replacement with a 27-mm St Jude
A
50 m
Endomyocardial biopsy sample (A) and excised aortic value cusp (B) Showing interstitial neutrophils (A, arrow) and focal degeneration and subendocardial macrophage activation (B, arrow) (haematoxilin-eosin ⫻20).
Medical prosthesis. The aortic valve had gross perforations, and focal fibrosal and mucoid degeneration and subendocardial macrophage infiltration on histological examination (figure, B). No micro-organisms or microthrombi characteristic of endocarditis were found in the surgical specimen (gram stains, acid-fast satins, and Grocott stains were negative). Postoperatively, the patient’s left ventricular function improved under tapered steroid therapy. When last seen in August, 2003, he had no complaints and his LVEDD was 62 mm. Sweet’s syndrome is characterised by the sudden onset of fever, neutrophilia, and rash1 (erythematous, welldemarcated papules and plaques with dense white cell infiltrates).2 Associated conditions include malignancies, infections, drug reactions, and autoimmune diseases.3 Cardiomegaly and acute valvulitis are also complications of Sweet’s syndrome.4,5 In addition to the usual extracutaneous manifestations, cardiac involvement can cause problems in patients with Sweet’s syndrome. We thank Dr Chalid Assaf for diagnosing Sweet’s syndrome and referring this patient to us.
References 1
Lancet 2003; 362: 1374 Department of Cardiology and Pneumology (M Dorenkamp MD, U Weikert MD, P L Schwimmbeck MD, A J Morguet MD), Benjamin Franklin University Hospital, 12200 Berlin, and Department of Pathology (R Meyer MD), German Heart Institute, 13353 Berlin, Germany Correspondence to: Dr Andreas J Morguet (e-mail: [email protected])
1374
B
2
3 4 5
Sweet RD. An acute febrile neutrophilic dermatosis. Br J Dermatol 1964; 139: 349–56. Delabie J, De Wolf-Peeters C, Mooren M, Karien K, Roskams T, Desmet V. Histiocytes in Sweet’s syndrome. Br J Dermatol 1991; 124: 348–53. Callen JP. Neutrophilic dermatoses. Dermatol Clin 2002; 20: 409–19. Shimizu K. Neutrophilic infiltration of myocardium in a patient with myelodysplastic syndrome. Am J Hematol 1998; 58: 337–38. Hayashi I, Hosoda Y, Kawasaki S, Yamamoto T, Dohi S, Kawai S. Aortic and mitral valve replacement in a patient with acute neutrophilic dermatosis (Sweet’s syndrome): report of a case. Surg Today 2001; 31: 810–13.
THE LANCET • Vol 362 • October 25, 2003 • www.thelancet.com
For personal use. Only reproduce with permission from The Lancet publishing Group.