NDT Plus (2010) 3: 494–495 doi: 10.1093/ndtplus/sfq101 Advance Access publication 8 June 2010
Images in Nephrology (Section Editor: G. H. Neild)
Glomeruloid haemangiomas associated with generalized oedema in a patient with atypical POEMS syndrome Keiji Kono1, Taro Masaki2, Yoko Funasaka2, Chikako Nishigori2 and Masafumi Fukagawa1,3 1
Division of Nephrology and Kidney Center, Kobe University School of Medicine, Kobe, Japan, 2Division of Dermatology, Kobe University School of Medicine, Kobe, Japan and 3Division of Nephrology and Metabolism, Tokai University School of Medicine, Isehara, Japan Correspondence and offprint requests to: Masafumi Fukagawa; E-mail: [email protected]
Keywords: glomeruloid haemangioma; POEMS syndrome
A 55-year-old woman was admitted for evaluation of progressive generalized oedema and mild kidney dysfunction (serum creatinine 1.12 mg/dL; blood urea nitrogen 45 mg/dL). On admission, generalized oedema, transudative pleural effusion, collapsed inferior vena cava and low fractional excretion of sodium (0.14%) suggested severe volume depletion due to plasma extravasation; however, its aetiology could not be attributed to decreased colloid osmotic pressure (serum total protein 6.4 g/dL). Further examination of the patient revealed hepatosplenomegaly, slowing of nerve conduction velocities, albuminocytological dissociation in the cerebrospinal fluid, and multiple cherry-red papules on the neck and chest (Figure 1). These features were suggestive of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome, also called osteosclerotic myeloma or Crow–Fukase syndrome. However, this case did not entirely fulfil the diagnostic criteria because monoclonal plasma cell disorders were not detected [1]. The skin biopsy revealed lesions consistent with glomeruloid haemangiomas (Figure 2), which is considered to be a specific marker for POEMS syndrome [2]. Furthermore, the
serum level of vascular endothelial growth factor (VEGF), which is involved in the pathogenesis of POEMS syndrome [1], was extremely elevated to 16 300 pg/mL. These findings led us to make a diagnosis of atypical POEMS syndrome and speculate that microvascular hyperpermeability induced by excess VEGF resulted in generalized oedema and effusions associated with severe volume depletion [3,4]. In patients with oedema and effusions of unknown cause, one should consider the possibility of disorders associated with VEGF overproduction including POEMS syndrome. References 1. Dispenzieri A. POEMS syndrome. Blood Rev 2007; 21: 285–299 2. Tsai CY, Lai CH, Chan HL et al. Glomeruloid hemangioma—a specific cutaneous marker of POEMS syndrome. Int J Dermatol 2001; 40: 403–406 3. Dvorak HF, Brown LF, Detmar M et al. Vascular permeability factor/ vascular endothelial growth factor, microvascular hyperpermeability, and angiogenesis. Am J Pathol 1995; 146: 1029–1039 4. Morizane R, Sasamura H, Minakuchi H et al. A case of atypical POEMS syndrome without polyneuropathy. Eur J Haematol 2008; 80: 452–455 Received for publication: 21.4.10; Accepted in revised form: 18.5.10
© The Author 2010. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For permissions, please e-mail: [email protected]
A specific marker for POEMS syndrome
495
Fig. 1. Multiple eruptive cherry-red papules on the neck.
Fig. 2. (a) Dermal lesions showing vascular structures with capillary loops, resembling renal glomeruli (haematoxylin–eosin stain). (b) Endothelial cells stained for CD 31.
Copyright of NDT Plus is the property of Oxford University Press / UK and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Images in Nephrology (Section Editor: G. H. Neild)
Glomeruloid haemangiomas associated with generalized oedema in a patient with atypical POEMS syndrome Keiji Kono1, Taro Masaki2, Yoko Funasaka2, Chikako Nishigori2 and Masafumi Fukagawa1,3 1
Division of Nephrology and Kidney Center, Kobe University School of Medicine, Kobe, Japan, 2Division of Dermatology, Kobe University School of Medicine, Kobe, Japan and 3Division of Nephrology and Metabolism, Tokai University School of Medicine, Isehara, Japan Correspondence and offprint requests to: Masafumi Fukagawa; E-mail: [email protected]
Keywords: glomeruloid haemangioma; POEMS syndrome
A 55-year-old woman was admitted for evaluation of progressive generalized oedema and mild kidney dysfunction (serum creatinine 1.12 mg/dL; blood urea nitrogen 45 mg/dL). On admission, generalized oedema, transudative pleural effusion, collapsed inferior vena cava and low fractional excretion of sodium (0.14%) suggested severe volume depletion due to plasma extravasation; however, its aetiology could not be attributed to decreased colloid osmotic pressure (serum total protein 6.4 g/dL). Further examination of the patient revealed hepatosplenomegaly, slowing of nerve conduction velocities, albuminocytological dissociation in the cerebrospinal fluid, and multiple cherry-red papules on the neck and chest (Figure 1). These features were suggestive of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome, also called osteosclerotic myeloma or Crow–Fukase syndrome. However, this case did not entirely fulfil the diagnostic criteria because monoclonal plasma cell disorders were not detected [1]. The skin biopsy revealed lesions consistent with glomeruloid haemangiomas (Figure 2), which is considered to be a specific marker for POEMS syndrome [2]. Furthermore, the
serum level of vascular endothelial growth factor (VEGF), which is involved in the pathogenesis of POEMS syndrome [1], was extremely elevated to 16 300 pg/mL. These findings led us to make a diagnosis of atypical POEMS syndrome and speculate that microvascular hyperpermeability induced by excess VEGF resulted in generalized oedema and effusions associated with severe volume depletion [3,4]. In patients with oedema and effusions of unknown cause, one should consider the possibility of disorders associated with VEGF overproduction including POEMS syndrome. References 1. Dispenzieri A. POEMS syndrome. Blood Rev 2007; 21: 285–299 2. Tsai CY, Lai CH, Chan HL et al. Glomeruloid hemangioma—a specific cutaneous marker of POEMS syndrome. Int J Dermatol 2001; 40: 403–406 3. Dvorak HF, Brown LF, Detmar M et al. Vascular permeability factor/ vascular endothelial growth factor, microvascular hyperpermeability, and angiogenesis. Am J Pathol 1995; 146: 1029–1039 4. Morizane R, Sasamura H, Minakuchi H et al. A case of atypical POEMS syndrome without polyneuropathy. Eur J Haematol 2008; 80: 452–455 Received for publication: 21.4.10; Accepted in revised form: 18.5.10
© The Author 2010. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For permissions, please e-mail: [email protected]
A specific marker for POEMS syndrome
495
Fig. 1. Multiple eruptive cherry-red papules on the neck.
Fig. 2. (a) Dermal lesions showing vascular structures with capillary loops, resembling renal glomeruli (haematoxylin–eosin stain). (b) Endothelial cells stained for CD 31.
Copyright of NDT Plus is the property of Oxford University Press / UK and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
