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10.1097/DAD.0000000000000234

http://scihub22266oqcxt.onion/10.1097/DAD.0000000000000234
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C4405909!4405909!25893747
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suck abstract from ncbi


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pmid25893747      Am+J+Dermatopathol 2015 ; 37 (5): 349-59
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  • THE ROLE OF THE SKIN BIOPSY IN THE DIAGNOSIS OF ATYPICAL HEMOLYTIC UREMIC SYNDROME #MMPMID25893747
  • Magro CM; Momtahen S; Mulvey JJ; Yassin AH; Kaplan RB; Laurence JC
  • Am J Dermatopathol 2015[May]; 37 (5): 349-59 PMID25893747show ga
  • Introduction: Atypical hemolytic uremic syndrome (aHUS) is a prototypic thrombotic microangiopathy attributable to complement dysregulation. In the absence of complement inhibition, progressive clinical deterioration occurs. We postulated that a biopsy of normal skin could corroborate the diagnosis of aHUS via the demonstration of vascular deposits of C5b-9. Materials and methods: Biopsies of normal skin from 22 patients with and without aHUS were processed for routine light microscopy as well as immunofluorescent studies. An assessment was made for vascular C5b-9 deposition immunohistochemically and by immunofluorescence. The biopsies were obtained primarily from the forearm and or deltoid. Results: Patients with classic features of atypical HUS showed insidious microvascular changes including loose luminal platelet thrombi except in two patients in whom a striking thrombogenic vasculopathy was apparent in biopsied digital ulcers. Extensive microvascular deposits of the membrane attack complex (MAC)/ C5b-9 were identified excluding one patient in whom eculizumab was initiated prior to biopsy. In 5 of the 7 patients where follow-up was available, the patients exhibited an excellent treatment response to eculizumab. Patients without diagnostic clinical features of atypical HUS failed to show significant vascular deposits of complement except two patients with TTP including one in whom a Factor H mutation was identified. Conclusion: In a clinical setting where aHUS is an important diagnostic consideration, extensive microvascular deposition of C5b-9 supports the diagnosis of either aHUS or a subset of TTP patients with concomitant complement dysregulation; significant vascular C5b-9 deposition predicts clinical responsiveness to eculizumab.
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