Glomeruloid Hemangioma A Distinctive Cutaneous Lesion of Multicentric Castlemanʼs Disease Associated with POEMS Syndrome

Chan, John K. C.; Fletcher, C. D. M.; Hicklin, Gregory A.; Rosaí, Juan

doi:10.1097/00000478-199011000-00005

Cited by 196 publications

(136 citation statements)

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“…As a consequence of the glomerular enlargement, capillary lumens became narrow and were surrounded by mature and immature endothelia, which were documented by using immunohistochemical analysis for CD34-positive cells. The capillary proliferation in the glomeruli may due to higher serum level of VEGF, because glomeruloid capillary haemangioma as capillary proliferation of the skin found in POEMS syndrome is suspected to be due to the higher serum level of VEGF and IL-6 (Chan et al 1990). …”

Section: Discussionmentioning

confidence: 99%

A Patient with POEMS Syndrome: The Pathology of Glomerular Microangiopathy

Nakamura

Nishimura

Terano

et al. 2013

Tohoku J. Exp. Med.

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POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) is potentially fatal multisystem disorder but its renal complication has often been overlooked because renal involvement is not necessarily included in the diagnostic criteria of POEMS. This report documents the patient with POEMS syndrome with long-term renal histopathological changes evaluated by renal biopsy. A 32-year-old Japanese woman presented with symptoms consistent with POEMS syndrome associated with proteinuria and IgA-λ type monoclonal gammopathy. Initial renal biopsy for confirmation of diagnosis revealed the proliferation of glomerular capillary loops located in the expanded mesangial matrices associated with glomerular enlargement. Electron microscopy examination of the renal biopsy revealed the presence of double contoured glomerular basement membrane containing peculiar fibrillary structure. The patient was therefore initially diagnosed as membranoproliferative glomerulonephritis (MPGN)-like lesion without any significant immunoglobulins and complements deposition. The patient was subsequently admitted to hospital on five occasions due to renal dysfunction and anasarca for the next four years of her clinical course. The severity of anasarca was correlated mainly with serum titer of vascular endothelial growth factor (VEGF) during this period. Acute renal failure occurred at the last admission and the second biopsy was performed. An increased mesangial matrix and frequent global sclerosis of the glomeruli with arteriolosclerosis was noted in this second biopsy compared to the first one. These findings of renal biopsies suggest that the glomerular microangiopathy of POEMS syndrome may occur in the context of systemic capillary leak syndrome superimposed on chronic endothelial injury.

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Section: Discussionmentioning

confidence: 99%

A Patient with POEMS Syndrome: The Pathology of Glomerular Microangiopathy

Nakamura

Nishimura

Terano

et al. 2013

Tohoku J. Exp. Med.

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“…In line with these observations, immunochemical staining did not show clonal immunoglobulin deposits in our case. Albeit reported in few other patients [6,15], glomeruloid hemangiomas are considered POEMS-specific [3,14]. Usually, those hemangiomas are limited to the skin.…”

Section: Discussionmentioning

confidence: 99%

Intracranial hemangiomas in a patient with POEMS syndrome

et al. 2009

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POEMS syndrome is a rare multi-system disease with typical features of polyneuropathy, organomegaly, endocrinopathy, monoclonal plasmaproliferative disorder and skin changes.We describe a 44 year-old woman with polyneuropathy, hepatomegaly, IgA lambdaplasmacytoma, thrombocytosis, papilledema with elevated protein levels in cerebrospinal fluid and multiple cutaneous hemangiomas who was diagnosed with three intracranial lesions.Histology revealed capillary hemangiomas, one of them displaying partially glomeruloid features.

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“…On histological examination, mature capillary angiomas are frequently described, but immature or cavernous angiomas may also be observed [2,11,13]. Recently Chan et al [14] and Rongioletti et al [2] described the histological features of glomeruloid haemangioma which can be considered as a histological marker of POEMS syndrome. Different types of angiomas may be found in the same patient and in the same lesion, which may correspond to different stages of development; tufted angioma may differentiate into glomeruloid or into cherry-type haemangioma [2].…”

Section: Discussionmentioning

confidence: 99%

“…No specific auto-antibody activity of the M protein has be demonstrated and its role is controversial [1,14,15]. Gherardi et al [15] have outlined the role of the pro-inflammatory cytokines IL-6, IL-1 and TNF-·, which are unbalanced by their antagonist (TGF-ß).…”

Section: Discussionmentioning

confidence: 99%

POEMS Syndrome Revealed by Multiple Glomeruloid Angiomas

et al. 2002

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POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin disorders) is a rare multisystemic disease associated with plasma cell dyscrasia. A 68-year-old woman with chronic renal insufficiency and arterial hypertension included in her medical history was admitted to the hospital with confusion, somnolence and asthenia. She presented ascites, hepatosplenomegaly, leg oedema, distal dysesthesias, leuconychia and multiple nodular purple red angiomas on the trunk, upper limbs and fingers. Hypothyroidism was revealed in the laboratory investigations and monoclonal IgG peak in immunoelectrophoresis. Electromyography showed both demyelinisating and axonal degenerative neuropathy. The diagnosis of POEMS syndrome was based on the dermatopathological examination of a cutaneous angioma; histology revealed features of glomeruloid angioma, a specific marker of this syndrome.

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Glomeruloid Hemangioma A Distinctive Cutaneous Lesion of Multicentric Castlemanʼs Disease Associated with POEMS Syndrome

Cited by 196 publications

References 0 publications

A Patient with POEMS Syndrome: The Pathology of Glomerular Microangiopathy

A Patient with POEMS Syndrome: The Pathology of Glomerular Microangiopathy

Intracranial hemangiomas in a patient with POEMS syndrome

POEMS Syndrome Revealed by Multiple Glomeruloid Angiomas

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