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Published in: Current Dermatology Reports 4/2019

01-12-2019 | Vasculitis | Hospital-Based Dermatology (L Guggina and A Zhou, Section Editors)

An Approach to Retiform Purpura for the Inpatient Dermatologist

Authors: Abeer Alsarheed, Gabriela A. Cobos, Christine M. Cornejo, Ruth Ann Vleugels, Joseph F. Merola

Published in: Current Dermatology Reports | Issue 4/2019

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Abstract

Purpose of Review

Retiform purpura is a potentially ominous cutaneous sign that develops secondary to vascular compromise by either vasculitis or a vasculopathic disorder. The wide array of associated complications mandate a thorough investigation in a timely fashion. This review provides an overview of the frequent causes of retiform purpura in hospitalized adult patients and proposes a diagnostic approach for the inpatient dermatologist.

Recent Findings

Early recognition of the primary cutaneous morphology in retiform purpura is especially important as this can help generate a focused differential and workup plan. A high-yield skin biopsy result warrants a proper site selection and an adequate depth. Further workup and treatment depend on the underlying pathogenic process. For patients with underlying systemic vasculitis, rituximab is emerging as an effective treatment. Rivaroxaban was found to have excess risk (increased thromboembolic and bleeding events) and no benefit when compared to warfarin in a trial of high-risk anti-phospholipid syndrome patients.

Summary

The differential diagnosis of retiform purpura is broad and a systematic approach is required to identify and treat the underlying cause.
Literature
1.
go back to reference Piette WW. The differential diagnosis of purpura from a morphologic perspective. Adv Dermatol. 1994;9:3–23 discussion 4.PubMed Piette WW. The differential diagnosis of purpura from a morphologic perspective. Adv Dermatol. 1994;9:3–23 discussion 4.PubMed
7.
go back to reference Tietge UJ, Schmidt HH, Jackel E, Trautwein C, Manns MP. Low molecular weight heparin-induced skin necrosis occurring distant from injection sites and without thrombocytopenia. J Intern Med. 1998;243(4):313–5.CrossRef Tietge UJ, Schmidt HH, Jackel E, Trautwein C, Manns MP. Low molecular weight heparin-induced skin necrosis occurring distant from injection sites and without thrombocytopenia. J Intern Med. 1998;243(4):313–5.CrossRef
13.
go back to reference Pinto-Almeida T, Caetano M, Sanches M, Selores M. Cutaneous manifestations of antiphospholipid syndrome: a review of the clinical features, diagnosis and management. Acta Reumatol Port. 2013;38(1):10–8.PubMed Pinto-Almeida T, Caetano M, Sanches M, Selores M. Cutaneous manifestations of antiphospholipid syndrome: a review of the clinical features, diagnosis and management. Acta Reumatol Port. 2013;38(1):10–8.PubMed
22.
go back to reference • Pengo V, Denas G, Zoppellaro G, Jose SP, Hoxha A, Ruffatti A, et al. Rivaroxaban vs warfarin in high-risk patients with antiphospholipid syndrome. Blood. 2018;132(13):1365–71. https://doi.org/10.1182/blood-2018-04-848333Randomized open-label multicenter noninferiority study which found rivaroxaban to have excess risk and no benefit when compared to warfarin in patients with APLS.CrossRefPubMed • Pengo V, Denas G, Zoppellaro G, Jose SP, Hoxha A, Ruffatti A, et al. Rivaroxaban vs warfarin in high-risk patients with antiphospholipid syndrome. Blood. 2018;132(13):1365–71. https://​doi.​org/​10.​1182/​blood-2018-04-848333Randomized open-label multicenter noninferiority study which found rivaroxaban to have excess risk and no benefit when compared to warfarin in patients with APLS.CrossRefPubMed
31.
go back to reference Hasegawa M, Sugiyama S. Apheresis in the treatment of cholesterol embolic disease. Ther Apher Dial. 2003;7(4):435–8.CrossRef Hasegawa M, Sugiyama S. Apheresis in the treatment of cholesterol embolic disease. Ther Apher Dial. 2003;7(4):435–8.CrossRef
37.
go back to reference Ghahramani G. Levamisole-associated vasculopathy and vasculitis. In: Misha Rosenbach, Karolyn A. Wanat, Micheletti RG, editors. Inpatient Dermatology Springer International Publishing; 2018. Ghahramani G. Levamisole-associated vasculopathy and vasculitis. In: Misha Rosenbach, Karolyn A. Wanat, Micheletti RG, editors. Inpatient Dermatology Springer International Publishing; 2018.
49.
go back to reference Wanat KA. Calciphylaxis. In: Misha Rosenbach, Karolyn A. Wanat, Micheletti RG, editors. Inpatient dermatology Springer International Publishing; 2018. Wanat KA. Calciphylaxis. In: Misha Rosenbach, Karolyn A. Wanat, Micheletti RG, editors. Inpatient dermatology Springer International Publishing; 2018.
55.
go back to reference Evaluation of Vitamin K supplementation for calcific uremic arteriolopathy. Evaluation of Vitamin K supplementation for calcific uremic arteriolopathy.
56.
go back to reference A Phase 3 Clinical trial of intravenous sodium thiosulfate in acute calciphylaxis patients. A Phase 3 Clinical trial of intravenous sodium thiosulfate in acute calciphylaxis patients.
62.
go back to reference Mahr A, Guillevin L, Poissonnet M, Ayme S. Prevalences of polyarteritis nodosa, microscopic polyangiitis, Wegener’s granulomatosis, and Churg-Strauss syndrome in a French urban multiethnic population in 2000: a capture-recapture estimate. Arthritis Rheum. 2004;51(1):92–9. https://doi.org/10.1002/art.20077.CrossRefPubMed Mahr A, Guillevin L, Poissonnet M, Ayme S. Prevalences of polyarteritis nodosa, microscopic polyangiitis, Wegener’s granulomatosis, and Churg-Strauss syndrome in a French urban multiethnic population in 2000: a capture-recapture estimate. Arthritis Rheum. 2004;51(1):92–9. https://​doi.​org/​10.​1002/​art.​20077.CrossRefPubMed
65.
go back to reference Pagnoux C, Seror R, Henegar C, Mahr A, Cohen P, Le Guern V, et al. Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database. Arthritis Rheum. 2010;62(2):616–26. https://doi.org/10.1002/art.27240.CrossRefPubMed Pagnoux C, Seror R, Henegar C, Mahr A, Cohen P, Le Guern V, et al. Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database. Arthritis Rheum. 2010;62(2):616–26. https://​doi.​org/​10.​1002/​art.​27240.CrossRefPubMed
72.
go back to reference De Groot K, Rasmussen N, Bacon PA, Tervaert JW, Feighery C, Gregorini G, et al. Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2005;52(8):2461–9. https://doi.org/10.1002/art.21142.CrossRefPubMed De Groot K, Rasmussen N, Bacon PA, Tervaert JW, Feighery C, Gregorini G, et al. Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2005;52(8):2461–9. https://​doi.​org/​10.​1002/​art.​21142.CrossRefPubMed
76.
go back to reference Comarmond C, Pagnoux C, Khellaf M, Cordier JF, Hamidou M, Viallard JF, et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort. Arthritis Rheum. 2013;65(1):270–81. https://doi.org/10.1002/art.37721.CrossRefPubMed Comarmond C, Pagnoux C, Khellaf M, Cordier JF, Hamidou M, Viallard JF, et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort. Arthritis Rheum. 2013;65(1):270–81. https://​doi.​org/​10.​1002/​art.​37721.CrossRefPubMed
78.
go back to reference • Teixeira V, Mohammad AJ, Jones RB, Smith R, Jayne D. Efficacy and safety of rituximab in the treatment of eosinophilic granulomatosis with polyangiitis. RMD Open. 2019;5(1):e000905. https://doi.org/10.1136/rmdopen-2019-000905Rituximab demonstrated efficacy and a steroid sparing effect in EGPA, but relapses occurred despite maintenance treatment. ANCA-positive patients had longer relapse-free survival time and a shorter time to remission.CrossRefPubMedPubMedCentral • Teixeira V, Mohammad AJ, Jones RB, Smith R, Jayne D. Efficacy and safety of rituximab in the treatment of eosinophilic granulomatosis with polyangiitis. RMD Open. 2019;5(1):e000905. https://​doi.​org/​10.​1136/​rmdopen-2019-000905Rituximab demonstrated efficacy and a steroid sparing effect in EGPA, but relapses occurred despite maintenance treatment. ANCA-positive patients had longer relapse-free survival time and a shorter time to remission.CrossRefPubMedPubMedCentral
79.
go back to reference • Wechsler ME, Akuthota P, Jayne D, Khoury P, Klion A, Langford CA, et al. Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis. N Engl J Med. 2017;376(20):1921–32. https://doi.org/10.1056/NEJMoa1702079A double-blind phase 3 trial demonstrated that mepolizumab in EGPA resulted in more weeks in remission and a higher overall proportion of remission than did placebo.CrossRefPubMedPubMedCentral • Wechsler ME, Akuthota P, Jayne D, Khoury P, Klion A, Langford CA, et al. Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis. N Engl J Med. 2017;376(20):1921–32. https://​doi.​org/​10.​1056/​NEJMoa1702079A double-blind phase 3 trial demonstrated that mepolizumab in EGPA resulted in more weeks in remission and a higher overall proportion of remission than did placebo.CrossRefPubMedPubMedCentral
82.
go back to reference Brouet JC, Clauvel JP, Danon F, Klein M, Seligmann M. Biologic and clinical significance of cryoglobulins. A report of 86 cases. Am J Med. 1974;57(5):775–88.CrossRef Brouet JC, Clauvel JP, Danon F, Klein M, Seligmann M. Biologic and clinical significance of cryoglobulins. A report of 86 cases. Am J Med. 1974;57(5):775–88.CrossRef
85.
go back to reference Carlson JA, Ng BT, Chen KR. Cutaneous vasculitis update: diagnostic criteria, classification, epidemiology, etiology, pathogenesis, evaluation and prognosis. Am J Dermatopathol. 2005;27(6):504–28.CrossRef Carlson JA, Ng BT, Chen KR. Cutaneous vasculitis update: diagnostic criteria, classification, epidemiology, etiology, pathogenesis, evaluation and prognosis. Am J Dermatopathol. 2005;27(6):504–28.CrossRef
88.
go back to reference Cozzani E, Gasparini G, Papini M, Burlando M, Drago F, Parodi A. Vasculitis associated with connective tissue diseases. G Ital Dermatol Venereol. 2015;150(2):221–32.PubMed Cozzani E, Gasparini G, Papini M, Burlando M, Drago F, Parodi A. Vasculitis associated with connective tissue diseases. G Ital Dermatol Venereol. 2015;150(2):221–32.PubMed
91.
go back to reference Doyle MK, Cuellar ML. Drug-induced vasculitis. Expert Opin Drug Saf. 2003;2(4):401–9.CrossRef Doyle MK, Cuellar ML. Drug-induced vasculitis. Expert Opin Drug Saf. 2003;2(4):401–9.CrossRef
92.
go back to reference Radic M, Martinovic Kaliterna D, Radic J. Drug-induced vasculitis: a clinical and pathological review. Neth J Med. 2012;70(1):12–7.PubMed Radic M, Martinovic Kaliterna D, Radic J. Drug-induced vasculitis: a clinical and pathological review. Neth J Med. 2012;70(1):12–7.PubMed
Metadata
Title
An Approach to Retiform Purpura for the Inpatient Dermatologist
Authors
Abeer Alsarheed
Gabriela A. Cobos
Christine M. Cornejo
Ruth Ann Vleugels
Joseph F. Merola
Publication date
01-12-2019
Publisher
Springer US
Published in
Current Dermatology Reports / Issue 4/2019
Electronic ISSN: 2162-4933
DOI
https://doi.org/10.1007/s13671-019-00281-2

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