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Staphylococcus cohnii as a cause of multiple brain abscesses in Weber–Christian disease
Journal of the Neurological Sciences 238 (2005) 97 – 100 www.elsevier.com/locate/jns
Short communication
Staphylococcus cohnii as a cause of multiple brain abscesses in Weber–Christian disease Satoshi Yamashita a,*, Kiminobu Yonemura a, Ryoko Sugimoto a, Makoto Tokunaga a, Makoto Uchino b a
Department of Neurology, Kumamoto Medical Center, National Hospital Organization, 1-5 Ninomaru, Kumamoto 860-0008, Japan Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, 1-1-1 Honjo, Kumamoto 860-0811, Japan
b
Received 30 December 2004; received in revised form 2 May 2005; accepted 27 June 2005 Available online 28 July 2005
Abstract We report a patient with multiple brain abscesses due to Staphylococcus cohnii. While these brain abscesses markedly responded to the antibiotics, this patient was subsequently suffered from subcutaneous inflammatory nodules in the adipose tissue, which diagnosed him as having Weber – Christian disease (WCD). This is the first report that subcutaneous inflammatory nodules in the adipose tissue, which lead the diagnosis of WCD, followed multiple brain abscesses. To our knowledge, S. cohnii has not yet been reported to cause multiple brain abscesses in humans. Although the etiology of WCD is unknown, an immune mechanism has been implicated in the pathogenesis. Therefore, we should notice that patients with WCD could be immunocompromised hosts with a higher risk to suffer from severe opportunistic infections. D 2005 Elsevier B.V. All rights reserved. Keywords: Weber – Christian disease; Panniculitis; Multiple brain abscesses; Staphylococcus cohnii; Subcutaneous inflammatory nodules; Soluble interleukin2 receptor
1. Introduction Weber –Christian disease (WCD) is a rare idiopathic systemic disease, characterized by recurrent subcutaneous inflammatory nodules in the adipose tissue with constitutional symptoms such as fever, general fatigue, polyarthralgia and polymyalgia. Although central or peripheral neurologic changes are present in one third of these patients, these symptoms have not been specifically emphasized previously [1]. We report the first patient with multiple brain abscesses due to Staphylococcus cohnii. While these brain abscesses markedly responded to the antibiotics, this patient was subsequently suffered from subcutaneous inflammatory
* Corresponding author. Present address: Department of Neurology, Columbia University, 650 West 168th Street, BB-320D, New York, NY 10032, USA. Tel.: +81 96 353 6501; fax: +81 88 642 1727. E-mail address: [email protected] (S. Yamashita). 0022-510X/$ - see front matter D 2005 Elsevier B.V. All rights reserved. doi:10.1016/j.jns.2005.06.009
nodules in the adipose tissue, which diagnosed him as having WCD.
2. Case report A 52-year-old man was admitted because of high fever, headache and bilateral knee joint pain. He was a former office worker and had no history of contact with farm animals. He had been treated for diabetes mellitus with insulin for 20 years. He had a history of phlegmonous inflammation due to unknown cause. He had not received any treatments such as corticosteroids or immunosuppressive agents. On admission, body temperature was 38.0 -C and physical examination revealed neither superficial lymphadenopathy nor hepatosplenomegaly. The knee joints were slightly swollen with local heat on both sides. Neurologic examination revealed no evidence of neck stiffness, or Kernig’s sign. Laboratory data showed leuko-
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S. Yamashita et al. / Journal of the Neurological Sciences 238 (2005) 97 – 100
cytosis of 19,800/Al with 83% segment neutrophils, Creactive protein (CRP) elevated to 13.54 mg/dl, and elevated erythrocyte sedimentation rate of 85 mm/h. Serum levels of aspartate aminotransferase, alanine aminotransferase, lactate dehydrogenase, amylase, creatinine, and blood glucose were within normal limits. Rheumatoid factor, antinuclear and anti-DNA antibodies, myeloperoxidase antineutrophil cytoplasmic antibodies (MPO-ANCA), and proteinase-3 antineutrophil cytoplasmic antibodies (PR3-ANCA) were negative. Alpha1-antitrypsin, thyroid function tests, and tumor markers (carcinoembryonic antigen (CEA), squamous cell carcinoma related antigen (SCC), and carbonhydrate antigen 19-9 (CA19-9)) were also within normal limits. Cerebro-spinal fluid examination revealed 13 neutrophils/mm3, protein of 66 mg/dl, and glucose of 99 mg/dl with a plasma glucose of 197 mg/dl. His brain MRI showed several ring-enhancing left temporal lesions (Fig. 1A). Diffusion-weighted MRI (DWI) revealed iso-intensity in the mass lesions (Fig. 1B). Chest radiography, transthoracic echocardiography, and electrocardiogram revealed no findings indicating infectious endocarditis or emboligenic heart disease. Considering the patient’s physical examination and laboratory data, multiple brain abscesses were suspected as the likely diagnosis. After taking a series of blood for culture and susceptibility tests, he was started on piperacillin (4 g every 12 h i.v.) and ceftriaxone (2 g every 12 h i.v.), or imipenem/cilastatin (1 g every 12 h i.v.) and clindamycin (1.2 g every 12 h i.v.) for 12 weeks. The bacterial cultures taken from two independent vessels grew S. cohnii, which was susceptible to all the antibiotics we employed. His state gradually returned to nearly normal, and he no longer complained of headache. While follow-up brain MRI 4
Fig. 2. (A) CT shows the severe edema of the subcutaneous adipose tissue of the left thigh. (B) Chest CT displays a subcutaneous inflammatory nodule in the adipose tissue of precordia (arrow).
weeks after admission showed obvious improvement of brain masses (Fig. 1C, D), he began to notice the severe edema of the subcutaneous adipose tissue of left thigh (Fig. 2A) and subcutaneous nodules located on the precordia (Fig. 2B). Although the patient had been treated with the antibiotics, laboratory analyses still revealed the presence of leukocytosis and the elevation of CRP. Serum concentration of soluble interleukin-2 receptor (sIL-2R) was extremely high (2909 U/ml, normal range: 145– 520). To confirm the diagnosis, a biopsy from subcutaneous nodule was taken at 12 weeks after admission. It revealed subcutaneous inflammation, which suggested degenerated fat, lipophages, mild nuclear cell infiltration, and fibrosis. There was no evidence of infection, vasculitis, and malignancy. After WCD was diagnosed, steroid pulse therapy (methylprednisolone 1000 mg/day i.v., 3 days) was initiated and dramatically diminished the febrile swelling of the left thigh and subcutaneous nodules located on the precordia. The serum level of sIL-2R decreased to 589 U/ml and the count of white blood cells and CRP returned to the normal level. He was then transferred to another hospital for the continuation of treatment.
3. Discussion
Fig. 1. (A) Axial T1-weighted brain MRI shows multiple temporal mass lesions with ring enhancement with gadolinium enhancement. (B) Axial diffusion-weighted brain MRI shows iso-intensity in the mass lesions. (C, D) Follow-up MRI shows obvious resolution of multiple brain abscesses after the treatment of antibiotics.
The clinical syndrome known as WCD, idiopathic relapsing nodular panniculitis, was first described by Pfeifer in 1892 and established by Weber and Christian in the 1920s [2]. WCD is usually diagnosed based on the histological findings, which show lobular panniculitis with lipophagia in biopsy specimens from the nodules. However, the term of WCD sometimes has been used for cases associated with infections, drug reactions, pancreatic disease, connective tissue disease, traumatic and factitial episodes, coagulation, vascular disease, and metabolic disease [3]. In this case, the
S. Yamashita et al. / Journal of the Neurological Sciences 238 (2005) 97 – 100
other conditions considered for differential diagnosis were a cytophagic histiocytic panniculitis, a subcutaneous T-cell lymphoma causing panniculitis, or panniculitis with infections. However, we diagnosed histologically this patient as having WCD. Other differential diagnosis like alpha1antitrypsin deficiency panniculitis, cutaneous polyarteritis nodosa, eosinophilic fasciculitis, scleroderma panniculitis, lupus panniculitis, panniculitis with pancreatic disease and erythema nodosum were also ruled out by the patient’s physical examination and laboratory data. Aronson et al. described that various signs and symptoms such as nonspecific systemic complaints, thrombocytosis, or abdominal symptoms were followed by the onset of either fever and/or nodules [1]. With regard to central neurologic changes, terminal neurologic manifestation, including seizure and coma, were seen in many patients and resembled Reye’s syndrome because of liver dysfunction. Although some clinicians reported that multiple intracranial xanthogranuloma occasionally occurred in association with WCD [4,5], brain mass lesions observed in this patient were suggested to be abscesses because they were shrunk after antibiotical treatments alone without any chemotherapies. This is the first report that subcutaneous inflammatory nodules in the adipose tissue, which lead the diagnosis of WCD, followed multiple brain abscesses. In this patient, Diffusion-weighted MRI showed almost iso-intense signal in brain masses. DWI has occasionally been used to diagnose cerebral abscesses. Kim et al. displayed a markedly hyper-intense signal in brain abscesses, whereas necrotic or cystic brain tumors gave a hypointense signal [6]. Recent publications suggest that DWI and apparent diffusion coefficient (ADC) calculations can be helpful in establishing a differential diagnosis between abscess and necrotic or cystic brain tumors [7]. Since we did not estimate ADC calculation in this patient, it was impossible to differentiate accurately between abscess and necrotic or cystic brain tumors by MRI alone. Although the etiology of WCD is unknown, an immune mechanism has been implicated in the pathogenesis, and it is frequently associated with hematological abnormalities and autoimmune diseases [8]. Iwasaki et al. demonstrated that extremely high serum concentrations of interferon-gamma and sIL-2R in the patient with WCD returned to normal with cyclosporin A, which is a suppressor of T-cell reactions [9]. Our patient also showed the finding of high serum concentration of sIL-2R, which suggests that T-cell immune responses could be involved in the pathogenesis of this case. However, we cannot fully eliminate the possibility that diabetes mellitus could affect the immune system even though his blood glucose was regulated well by injection of insulin. Aronson et al. reported that three major factors associated with the onset or recurrence of panniculitis were trauma, drugs, and infection [1]. The relationship between infections and the onset of panniculitis is still unclear, but we could
99
speculate that both lymphokine and lipid-induced macrophage, which normal lymphocytes activated by infectious agents produced, might play an important role in the onset of WCD. Coagulase-negative staphylococcus species are frequently isolated from blood cultures, but S. cohnii is usually isolated from farm animals and rarely responsible for human systemic infections. Some authors reported cases of sepsis or meningitis in a neonate or cancer patient [10,11]. To our knowledge, S. cohnii has not yet been reported to cause multiple brain abscesses in humans. Szewczyk et al. showed that S. cohnii was found in the ward environment, and that it would certainly have been present on the skin of personnel and patients [12]. In terms of the source of S. cohnii infection, we could speculate that it might invade this patient transcutaneously because he had received regular outpatient treatment and been injected periodically recombinant insulin. Although coagulase-negative Staphylococcus sp. are also the most common blood culture contaminants [10], we can rule out a possibility of contamination of S. cohnii because a series of blood cultures were obtained from several independent vessels. The prognosis of WCD is extremely variable from short, selflimited, or intermittent disease episodes to persistent disease with fatal outcome. Infection including sepsis is reported to be one of the major causes of death in patients with the systemic histiocytic proliferation. Therefore, we should notice that patients with WCD could be immunocompromised hosts with a higher risk to suffer from severe opportunistic infections.
References [1] Aronson IK, West DP, Variakojis D, Malkinson FD, Wilson HD, Zeitz HJ. Fatal panniculitis. J Am Acad Dermatol 1985;12:535 – 51. [2] Steffen C. The men behind the eponym: Frederick Parkes Weber and Henry A. Christian: Weber – Christian disease. Am J Dermatopathol 2002;24:514 – 7. [3] White Jr JW, Winkelmann RK. Weber – Christian panniculitis: a review of 30 cases with this diagnosis. J Am Acad Dermatol 1998; 39:56 – 62. [4] Nagatani K, Waga S, Takeuchi J, Takebe Y, Hohda K, Hirano A. Multiple intracranial xanthogranulomas—case report. Neurol Med Chir (Tokyo) 1990;30:960 – 5. [5] Miyachi S, Kobayashi T, Takahashi T, Saito K, Hashizume Y, Sugita K. An intracranial mass lesion in systemic xanthogranulomatosis: case report. Neurosurgery 1990;27:822 – 6. [6] Kim YJ, Chang KH, Song IC, Kim HD, Seong SO, Kim YH, et al. Brain abscess and necrotic or cystic brain tumor: discrimination with signal intensity on diffusion-weighted MR imaging. AJR Am J Roentgenol 1998;171:1487 – 90. [7] Desprechins B, Stadnik T, Koerts G, Shabana W, Breucq C, Osteaux M. Use of diffusion-weighted MR imaging in differential diagnosis between intracerebral necrotic tumors and cerebral abscesses. AJNR Am J Neuroradiol 1999;20:1252 – 7. [8] Jimenez-Mazuecos J, Yebra-Bango M, Sanchez-Ruiz A, VillarrealGarcia-Lomas M. Weber – Christian disease associated with lupus anticoagulant and anticardiolipin antibodies. Rheumatology (Oxford) 1999;38:796 – 7.
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[9] Iwasaki T, Hamano T, Ogata A, Hashimoto N, Kakishita E. Successful treatment of a patient with febrile, lobular panniculitis (Weber – Christian disease) with oral cyclosporin A: implications for pathogenesis and therapy. Intern Med 1999;38:612 – 4. [10] Kaya IS, Gamberzade S, Toppare MF, Senses DA, Dilmen U, Kitapci F. Neonatal sepsis and meningitis due to Staphylococcus cohnii. J Pak Med Assoc 1996;46:43 – 4.
[11] Basaglia G, Moras L, Bearz A, Scalone S, Paoli PD. Staphylococcus cohnii septicaemia in a patient with colon cancer. J Med Microbiol 2003;52:101 – 2. [12] Szewczyk EM, Piotrowski A, Rozalska M. Predominant staphylococci in the intensive care unit of a paediatric hospital. J Hosp Infect 2000;45:145 – 54.
Short communication
Staphylococcus cohnii as a cause of multiple brain abscesses in Weber–Christian disease Satoshi Yamashita a,*, Kiminobu Yonemura a, Ryoko Sugimoto a, Makoto Tokunaga a, Makoto Uchino b a
Department of Neurology, Kumamoto Medical Center, National Hospital Organization, 1-5 Ninomaru, Kumamoto 860-0008, Japan Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, 1-1-1 Honjo, Kumamoto 860-0811, Japan
b
Received 30 December 2004; received in revised form 2 May 2005; accepted 27 June 2005 Available online 28 July 2005
Abstract We report a patient with multiple brain abscesses due to Staphylococcus cohnii. While these brain abscesses markedly responded to the antibiotics, this patient was subsequently suffered from subcutaneous inflammatory nodules in the adipose tissue, which diagnosed him as having Weber – Christian disease (WCD). This is the first report that subcutaneous inflammatory nodules in the adipose tissue, which lead the diagnosis of WCD, followed multiple brain abscesses. To our knowledge, S. cohnii has not yet been reported to cause multiple brain abscesses in humans. Although the etiology of WCD is unknown, an immune mechanism has been implicated in the pathogenesis. Therefore, we should notice that patients with WCD could be immunocompromised hosts with a higher risk to suffer from severe opportunistic infections. D 2005 Elsevier B.V. All rights reserved. Keywords: Weber – Christian disease; Panniculitis; Multiple brain abscesses; Staphylococcus cohnii; Subcutaneous inflammatory nodules; Soluble interleukin2 receptor
1. Introduction Weber –Christian disease (WCD) is a rare idiopathic systemic disease, characterized by recurrent subcutaneous inflammatory nodules in the adipose tissue with constitutional symptoms such as fever, general fatigue, polyarthralgia and polymyalgia. Although central or peripheral neurologic changes are present in one third of these patients, these symptoms have not been specifically emphasized previously [1]. We report the first patient with multiple brain abscesses due to Staphylococcus cohnii. While these brain abscesses markedly responded to the antibiotics, this patient was subsequently suffered from subcutaneous inflammatory
* Corresponding author. Present address: Department of Neurology, Columbia University, 650 West 168th Street, BB-320D, New York, NY 10032, USA. Tel.: +81 96 353 6501; fax: +81 88 642 1727. E-mail address: [email protected] (S. Yamashita). 0022-510X/$ - see front matter D 2005 Elsevier B.V. All rights reserved. doi:10.1016/j.jns.2005.06.009
nodules in the adipose tissue, which diagnosed him as having WCD.
2. Case report A 52-year-old man was admitted because of high fever, headache and bilateral knee joint pain. He was a former office worker and had no history of contact with farm animals. He had been treated for diabetes mellitus with insulin for 20 years. He had a history of phlegmonous inflammation due to unknown cause. He had not received any treatments such as corticosteroids or immunosuppressive agents. On admission, body temperature was 38.0 -C and physical examination revealed neither superficial lymphadenopathy nor hepatosplenomegaly. The knee joints were slightly swollen with local heat on both sides. Neurologic examination revealed no evidence of neck stiffness, or Kernig’s sign. Laboratory data showed leuko-
98
S. Yamashita et al. / Journal of the Neurological Sciences 238 (2005) 97 – 100
cytosis of 19,800/Al with 83% segment neutrophils, Creactive protein (CRP) elevated to 13.54 mg/dl, and elevated erythrocyte sedimentation rate of 85 mm/h. Serum levels of aspartate aminotransferase, alanine aminotransferase, lactate dehydrogenase, amylase, creatinine, and blood glucose were within normal limits. Rheumatoid factor, antinuclear and anti-DNA antibodies, myeloperoxidase antineutrophil cytoplasmic antibodies (MPO-ANCA), and proteinase-3 antineutrophil cytoplasmic antibodies (PR3-ANCA) were negative. Alpha1-antitrypsin, thyroid function tests, and tumor markers (carcinoembryonic antigen (CEA), squamous cell carcinoma related antigen (SCC), and carbonhydrate antigen 19-9 (CA19-9)) were also within normal limits. Cerebro-spinal fluid examination revealed 13 neutrophils/mm3, protein of 66 mg/dl, and glucose of 99 mg/dl with a plasma glucose of 197 mg/dl. His brain MRI showed several ring-enhancing left temporal lesions (Fig. 1A). Diffusion-weighted MRI (DWI) revealed iso-intensity in the mass lesions (Fig. 1B). Chest radiography, transthoracic echocardiography, and electrocardiogram revealed no findings indicating infectious endocarditis or emboligenic heart disease. Considering the patient’s physical examination and laboratory data, multiple brain abscesses were suspected as the likely diagnosis. After taking a series of blood for culture and susceptibility tests, he was started on piperacillin (4 g every 12 h i.v.) and ceftriaxone (2 g every 12 h i.v.), or imipenem/cilastatin (1 g every 12 h i.v.) and clindamycin (1.2 g every 12 h i.v.) for 12 weeks. The bacterial cultures taken from two independent vessels grew S. cohnii, which was susceptible to all the antibiotics we employed. His state gradually returned to nearly normal, and he no longer complained of headache. While follow-up brain MRI 4
Fig. 2. (A) CT shows the severe edema of the subcutaneous adipose tissue of the left thigh. (B) Chest CT displays a subcutaneous inflammatory nodule in the adipose tissue of precordia (arrow).
weeks after admission showed obvious improvement of brain masses (Fig. 1C, D), he began to notice the severe edema of the subcutaneous adipose tissue of left thigh (Fig. 2A) and subcutaneous nodules located on the precordia (Fig. 2B). Although the patient had been treated with the antibiotics, laboratory analyses still revealed the presence of leukocytosis and the elevation of CRP. Serum concentration of soluble interleukin-2 receptor (sIL-2R) was extremely high (2909 U/ml, normal range: 145– 520). To confirm the diagnosis, a biopsy from subcutaneous nodule was taken at 12 weeks after admission. It revealed subcutaneous inflammation, which suggested degenerated fat, lipophages, mild nuclear cell infiltration, and fibrosis. There was no evidence of infection, vasculitis, and malignancy. After WCD was diagnosed, steroid pulse therapy (methylprednisolone 1000 mg/day i.v., 3 days) was initiated and dramatically diminished the febrile swelling of the left thigh and subcutaneous nodules located on the precordia. The serum level of sIL-2R decreased to 589 U/ml and the count of white blood cells and CRP returned to the normal level. He was then transferred to another hospital for the continuation of treatment.
3. Discussion
Fig. 1. (A) Axial T1-weighted brain MRI shows multiple temporal mass lesions with ring enhancement with gadolinium enhancement. (B) Axial diffusion-weighted brain MRI shows iso-intensity in the mass lesions. (C, D) Follow-up MRI shows obvious resolution of multiple brain abscesses after the treatment of antibiotics.
The clinical syndrome known as WCD, idiopathic relapsing nodular panniculitis, was first described by Pfeifer in 1892 and established by Weber and Christian in the 1920s [2]. WCD is usually diagnosed based on the histological findings, which show lobular panniculitis with lipophagia in biopsy specimens from the nodules. However, the term of WCD sometimes has been used for cases associated with infections, drug reactions, pancreatic disease, connective tissue disease, traumatic and factitial episodes, coagulation, vascular disease, and metabolic disease [3]. In this case, the
S. Yamashita et al. / Journal of the Neurological Sciences 238 (2005) 97 – 100
other conditions considered for differential diagnosis were a cytophagic histiocytic panniculitis, a subcutaneous T-cell lymphoma causing panniculitis, or panniculitis with infections. However, we diagnosed histologically this patient as having WCD. Other differential diagnosis like alpha1antitrypsin deficiency panniculitis, cutaneous polyarteritis nodosa, eosinophilic fasciculitis, scleroderma panniculitis, lupus panniculitis, panniculitis with pancreatic disease and erythema nodosum were also ruled out by the patient’s physical examination and laboratory data. Aronson et al. described that various signs and symptoms such as nonspecific systemic complaints, thrombocytosis, or abdominal symptoms were followed by the onset of either fever and/or nodules [1]. With regard to central neurologic changes, terminal neurologic manifestation, including seizure and coma, were seen in many patients and resembled Reye’s syndrome because of liver dysfunction. Although some clinicians reported that multiple intracranial xanthogranuloma occasionally occurred in association with WCD [4,5], brain mass lesions observed in this patient were suggested to be abscesses because they were shrunk after antibiotical treatments alone without any chemotherapies. This is the first report that subcutaneous inflammatory nodules in the adipose tissue, which lead the diagnosis of WCD, followed multiple brain abscesses. In this patient, Diffusion-weighted MRI showed almost iso-intense signal in brain masses. DWI has occasionally been used to diagnose cerebral abscesses. Kim et al. displayed a markedly hyper-intense signal in brain abscesses, whereas necrotic or cystic brain tumors gave a hypointense signal [6]. Recent publications suggest that DWI and apparent diffusion coefficient (ADC) calculations can be helpful in establishing a differential diagnosis between abscess and necrotic or cystic brain tumors [7]. Since we did not estimate ADC calculation in this patient, it was impossible to differentiate accurately between abscess and necrotic or cystic brain tumors by MRI alone. Although the etiology of WCD is unknown, an immune mechanism has been implicated in the pathogenesis, and it is frequently associated with hematological abnormalities and autoimmune diseases [8]. Iwasaki et al. demonstrated that extremely high serum concentrations of interferon-gamma and sIL-2R in the patient with WCD returned to normal with cyclosporin A, which is a suppressor of T-cell reactions [9]. Our patient also showed the finding of high serum concentration of sIL-2R, which suggests that T-cell immune responses could be involved in the pathogenesis of this case. However, we cannot fully eliminate the possibility that diabetes mellitus could affect the immune system even though his blood glucose was regulated well by injection of insulin. Aronson et al. reported that three major factors associated with the onset or recurrence of panniculitis were trauma, drugs, and infection [1]. The relationship between infections and the onset of panniculitis is still unclear, but we could
99
speculate that both lymphokine and lipid-induced macrophage, which normal lymphocytes activated by infectious agents produced, might play an important role in the onset of WCD. Coagulase-negative staphylococcus species are frequently isolated from blood cultures, but S. cohnii is usually isolated from farm animals and rarely responsible for human systemic infections. Some authors reported cases of sepsis or meningitis in a neonate or cancer patient [10,11]. To our knowledge, S. cohnii has not yet been reported to cause multiple brain abscesses in humans. Szewczyk et al. showed that S. cohnii was found in the ward environment, and that it would certainly have been present on the skin of personnel and patients [12]. In terms of the source of S. cohnii infection, we could speculate that it might invade this patient transcutaneously because he had received regular outpatient treatment and been injected periodically recombinant insulin. Although coagulase-negative Staphylococcus sp. are also the most common blood culture contaminants [10], we can rule out a possibility of contamination of S. cohnii because a series of blood cultures were obtained from several independent vessels. The prognosis of WCD is extremely variable from short, selflimited, or intermittent disease episodes to persistent disease with fatal outcome. Infection including sepsis is reported to be one of the major causes of death in patients with the systemic histiocytic proliferation. Therefore, we should notice that patients with WCD could be immunocompromised hosts with a higher risk to suffer from severe opportunistic infections.
References [1] Aronson IK, West DP, Variakojis D, Malkinson FD, Wilson HD, Zeitz HJ. Fatal panniculitis. J Am Acad Dermatol 1985;12:535 – 51. [2] Steffen C. The men behind the eponym: Frederick Parkes Weber and Henry A. Christian: Weber – Christian disease. Am J Dermatopathol 2002;24:514 – 7. [3] White Jr JW, Winkelmann RK. Weber – Christian panniculitis: a review of 30 cases with this diagnosis. J Am Acad Dermatol 1998; 39:56 – 62. [4] Nagatani K, Waga S, Takeuchi J, Takebe Y, Hohda K, Hirano A. Multiple intracranial xanthogranulomas—case report. Neurol Med Chir (Tokyo) 1990;30:960 – 5. [5] Miyachi S, Kobayashi T, Takahashi T, Saito K, Hashizume Y, Sugita K. An intracranial mass lesion in systemic xanthogranulomatosis: case report. Neurosurgery 1990;27:822 – 6. [6] Kim YJ, Chang KH, Song IC, Kim HD, Seong SO, Kim YH, et al. Brain abscess and necrotic or cystic brain tumor: discrimination with signal intensity on diffusion-weighted MR imaging. AJR Am J Roentgenol 1998;171:1487 – 90. [7] Desprechins B, Stadnik T, Koerts G, Shabana W, Breucq C, Osteaux M. Use of diffusion-weighted MR imaging in differential diagnosis between intracerebral necrotic tumors and cerebral abscesses. AJNR Am J Neuroradiol 1999;20:1252 – 7. [8] Jimenez-Mazuecos J, Yebra-Bango M, Sanchez-Ruiz A, VillarrealGarcia-Lomas M. Weber – Christian disease associated with lupus anticoagulant and anticardiolipin antibodies. Rheumatology (Oxford) 1999;38:796 – 7.
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[9] Iwasaki T, Hamano T, Ogata A, Hashimoto N, Kakishita E. Successful treatment of a patient with febrile, lobular panniculitis (Weber – Christian disease) with oral cyclosporin A: implications for pathogenesis and therapy. Intern Med 1999;38:612 – 4. [10] Kaya IS, Gamberzade S, Toppare MF, Senses DA, Dilmen U, Kitapci F. Neonatal sepsis and meningitis due to Staphylococcus cohnii. J Pak Med Assoc 1996;46:43 – 4.
[11] Basaglia G, Moras L, Bearz A, Scalone S, Paoli PD. Staphylococcus cohnii septicaemia in a patient with colon cancer. J Med Microbiol 2003;52:101 – 2. [12] Szewczyk EM, Piotrowski A, Rozalska M. Predominant staphylococci in the intensive care unit of a paediatric hospital. J Hosp Infect 2000;45:145 – 54.