Skip to main content
SpringerLink
Log in

Vasculitis and Neutrophilic Dermatoses and Related Disorders

  • Living reference work entry
  • First Online:
Atlas of Dermatology, Dermatopathology and Venereology

  • 219 Accesses

Abstract

Vasculitis represents a group of conditions that are characterized by inflammatory involvement of vessels and are usually classified based on the caliber of the affected vessel. Their presentations are variable, ranging from palpable purpura to painful, nonhealing ulcerations. Neutrophilic dermatoses represent the group of conditions with diffuse neutrophilic infiltrate that are commonly associated with other inflammatory systemic conditions and neoplasms.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution

Institutional subscriptions

References

  • Arora A, Wetter DA, Gonzalez-Santiago TM, Davis MD, Lohse CM (2014) Incidence of leukocytoclastic vasculitis, 1996 to 2010: a population-based study in Olmsted County, Minnesota. Mayo Clin Proc 89(11):1515–1524

    PubMed  PubMed Central  Google Scholar 

  • Bennett ML, Jackson JM, Jorizzo JL, Fleischer AB Jr, White WL, Callen JP (2000) Pyoderma gangrenosum. A comparison of typical and atypical forms with an emphasis on time to remission. Case review of 86 patients from 2 institutions. Medicine (Baltimore) 79(1):37–46

    CAS  Google Scholar 

  • Broekaert SM, Boer-Auer A, Kerl K, Herrgott I, Schulz X, Bonsmann G, Brehler R, Metze D (2016) Neutrophilic epitheliotropism is a histopathological clue to neutrophilic urticarial dermatosis. Am J Dermatopathol 38(1):39–49

    PubMed  Google Scholar 

  • Chung SA, Seo P (2010) Microscopic polyangiitis. Rheum Dis Clin North Am 36(3):545–558

    PubMed  PubMed Central  Google Scholar 

  • Cohen PR (2007) Sweet’s syndrome – a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J Rare Dis 2:34

    PubMed  PubMed Central  Google Scholar 

  • Comfere NI, Macaron NC, Gibson LE (2007) Cutaneous manifestations of Wegener’s granulomatosis: a clinicopathologic study of 17 patients and correlation to antineutrophil cytoplasmic antibody status. J Cutan Pathol 34(10):739–747

    PubMed  Google Scholar 

  • Cottin V, Bel E, Bottero P, Dalhoff K, Humbert M, Lazor R, Sinico RA, Sivasothy P, Wechsler ME, Groh M, Marchand-Adam S, Khouatra C, Wallaert B, Taille C, Delaval P, Cadranel J, Bonniaud P, Prevot G, Hirschi S, Gondouin A, Dunogue B, Chatte G, Briault C, Pagnoux C, Jayne D, Guillevin L, Cordier JF (2017) Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): a study of 157 patients by the Groupe d’Etudes et de Recherche sur les Maladies Orphelines Pulmonaires and the European Respiratory Society Taskforce on eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Autoimmun Rev 16(1):1–9

    CAS  PubMed  Google Scholar 

  • Coutinho I, Pereira N, Gouveia M, Cardoso JC, Tellechea O (2015) Interstitial granulomatous dermatitis: a clinicopathological study. Am J Dermatopathol 37(8):614–619

    PubMed  Google Scholar 

  • Davis MD, Brewer JD (2004) Urticarial vasculitis and hypocomplementemic urticarial vasculitis syndrome. Immunol Allergy Clin North Am 24(2):183–213

    PubMed  Google Scholar 

  • De Virgilio A, Greco A, Magliulo G, Gallo A, Ruoppolo G, Conte M, Martellucci S, de Vincentiis M (2016) Polyarteritis nodosa: a contemporary overview. Autoimmun Rev 15(6):564–570

    PubMed  Google Scholar 

  • Demirkesen C (2017) Approach to cutaneous vasculitides with special emphasis on small vessel vasculitis: histopathology and direct immunofluorescence. Curr Opin Rheumatol 29(1):39–44

    CAS  PubMed  Google Scholar 

  • Giuggioli D, Manfredi A, Lumetti F, Sebastiani M, Ferri C (2015) Cryoglobulinemic vasculitis and skin ulcers. Our therapeutic strategy and review of the literature. Semin Arthritis Rheum 44(5):518–526

    CAS  PubMed  Google Scholar 

  • Groh M, Pagnoux C, Baldini C, Bel E, Bottero P, Cottin V, Dalhoff K, Dunogue B, Gross W, Holle J, Humbert M, Jayne D, Jennette JC, Lazor R, Mahr A, Merkel PA, Mouthon L, Sinico RA, Specks U, Vaglio A, Wechsler ME, Cordier JF, Guillevin L (2015) Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management. Eur J Intern Med 26(7):545–553

    PubMed  Google Scholar 

  • Hau E, Vignon Pennamen MD, Battistella M, Saussine A, Bergis M, Cavelier-Balloy B, Janier M, Cordoliani F, Bagot M, Rybojad M, Bouaziz JD (2014) Neutrophilic skin lesions in autoimmune connective tissue diseases: nine cases and a literature review. Medicine (Baltimore) 93(29):e346

    CAS  Google Scholar 

  • Heineke MH, Ballering AV, Jamin A, Ben Mkaddem S, Monteiro RC, Van Egmond M (2017) New insights in the pathogenesis of immunoglobulin A vasculitis (Henoch-Schonlein purpura). Autoimmun Rev 16(12):1246–1253

    CAS  PubMed  Google Scholar 

  • Hodge SJ, Callen JP, Ekenstam E (1987) Cutaneous leukocytoclastic vasculitis: correlation of histopathological changes with clinical severity and course. J Cutan Pathol 14(5):279–284

    CAS  PubMed  Google Scholar 

  • Jachiet M, Flageul B, Deroux A, Le Quellec A, Maurier F, Cordoliani F, Godmer P, Abasq C, Astudillo L, Belenotti P, Bessis D, Bigot A, Doutre MS, Ebbo M, Guichard I, Hachulla E, Heron E, Jeudy G, Jourde-Chiche N, Jullien D, Lavigne C, Machet L, Macher MA, Martel C, Melboucy-Belkhir S, Morice C, Petit A, Simorre B, Zenone T, Bouillet L, Bagot M, Fremeaux-Bacchi V, Guillevin L, Mouthon L, Dupin N, Aractingi S, Terrier B (2015) The clinical spectrum and therapeutic management of hypocomplementemic urticarial vasculitis: data from a French nationwide study of fifty-seven patients. Arthritis Rheumatol 67(2):527–534

    CAS  PubMed  Google Scholar 

  • Jenkins J, Babu K, Hsu-Hung E, Robinson-Bostom L, Kroumpouzos G (2011) ANCA-positive necrotizing vasculitis and thrombotic vasculopathy induced by levamisole-adulterated cocaine: a distinctive clinicopathologic presentation. J Am Acad Dermatol 65(1):e14–e16

    PubMed  Google Scholar 

  • Kazlouskaya V, Junkins-Hopkins JM (2018) Lymphocytes in Sweet syndrome: a potential diagnostic pitfall. J Cutan Pathol 45(3):217–222

    PubMed  Google Scholar 

  • Kern M, Shiver MB, Addis KM, Gardner JM (2017) Palisaded neutrophilic and granulomatous dermatitis/interstitial granulomatous dermatitis overlap: a striking clinical and histologic presentation with “burning rope sign” and subsequent mirror-image contralateral recurrence. Am J Dermatopathol 39(9):e141–e146

    PubMed  Google Scholar 

  • Lee JS, Loh TH, Seow SC, Tan SH (2007) Prolonged urticaria with purpura: the spectrum of clinical and histopathologic features in a prospective series of 22 patients exhibiting the clinical features of urticarial vasculitis. J Am Acad Dermatol 56(6):994–1005

    PubMed  Google Scholar 

  • Lindhaus C, Elsner P (2018) Granuloma faciale treatment: a systematic review. Acta Derm Venereol 98(1):14–18

    CAS  PubMed  Google Scholar 

  • Loricera J, Calvo-Rio V, Mata C, Ortiz-Sanjuan F, Gonzalez-Lopez MA, Alvarez L, Gonzalez-Vela MC, Armesto S, Fernandez-Llaca H, Rueda-Gotor J, Gonzalez-Gay MA, Blanco R (2014) Urticarial vasculitis in northern Spain: clinical study of 21 cases. Medicine (Baltimore) 93(1):53–60

    Google Scholar 

  • Maverakis E, Ma C, Shinkai K, Fiorentino D, Callen JP, Wollina U, Marzano AV, Wallach D, Kim K, Schadt C, Ormerod A, Fung MA, Steel A, Patel F, Qin R, Craig F, Williams HC, Powell F, Merleev A, Cheng MY (2018) Diagnostic criteria of ulcerative pyoderma gangrenosum: a Delphi Consensus of International Experts. JAMA Dermatol 154(4):461–466

    PubMed  Google Scholar 

  • Mazzaro C, Dal Maso L, Urraro T, Mauro E, Castelnovo L, Casarin P, Monti G, Gattei V, Zignego AL, Pozzato G (2016) Hepatitis B virus related cryoglobulinemic vasculitis: a multicentre open label study from the Gruppo Italiano di Studio delle Crioglobulinemie – GISC. Dig Liver Dis 48(7):780–784

    PubMed  Google Scholar 

  • Momen SE, Jorizzo J, Al-Niaimi F (2014) Erythema elevatum diutinum: a review of presentation and treatment. J Eur Acad Dermatol Venereol 28(12):1594–1602

    CAS  PubMed  Google Scholar 

  • Morimoto A, Chen KR (2016) Reappraisal of histopathology of cutaneous polyarteritis nodosa. J Cutan Pathol 43(12):1131–1138

    PubMed  Google Scholar 

  • Morris GW Jr, Mason BC, Harris Sprunger R, Hake Harris H, White LA, Patterson DA (2012) Levamisole-adulterated cocaine: a case series. J Am Board Fam Med 25(4):531–535

    PubMed  Google Scholar 

  • Muñoz-Vahos CH, Herrera-Uribe S, Arbeláez-Cortés Á, Jaramillo-Arroyave D, González-Naranjo LA, Vásquez-Duque G, Restrepo-Escobar M, Correa-Londoño LA, Arias-Restrepo LF, Vanegas-García AL (2018) Clinical profile of levamisole-adulterated cocaine-induced vasculitis/vasculopathy: a 30-case series. J Clin Rheumatol. https://doi.org/10.1097/RHU.0000000000000813

  • Ortonne N, Wechsler J, Bagot M, Grosshans E, Cribier B (2005) Granuloma faciale: a clinicopathologic study of 66 patients. J Am Acad Dermatol 53(6):1002–1009

    PubMed  Google Scholar 

  • Ostojic P, Jeremic IR (2017) Managing refractory cryoglobulinemic vasculitis: challenges and solutions. J Inflamm Res 10:49–54

    CAS  PubMed  PubMed Central  Google Scholar 

  • Ozen S (2017) The changing face of polyarteritis nodosa and necrotizing vasculitis. Nat Rev Rheumatol 13(6):381–386

    PubMed  Google Scholar 

  • Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, Buoncompagni A, Lazar C, Bilge I, Uziel Y, Rigante D, Cantarini L, Hilario MO, Silva CA, Alegria M, Norambuena X, Belot A, Berkun Y, Estrella AI, Olivieri AN, Alpigiani MG, Rumba I, Sztajnbok F, Tambic-Bukovac L, Breda L, Al-Mayouf S, Mihaylova D, Chasnyk V, Sengler C, Klein-Gitelman M, Djeddi D, Nuno L, Pruunsild C, Brunner J, Kondi A, Pagava K, Pederzoli S, Martini A, Ruperto N (2010) EULAR/PRINTO/PRES criteria for Henoch-Schonlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: final classification criteria. Ann Rheum Dis 69(5):798–806

    PubMed  Google Scholar 

  • Pagnoux C (2016) Updates in ANCA-associated vasculitis. Eur J Rheumatol 3(3):122–133

    PubMed  PubMed Central  Google Scholar 

  • Parker L, Shahar-Nissan K, Ashkenazi-Hoffnung L, Harel L, Amir J, Trivizki O, Bilavsky E (2017) Acute hemorrhagic edema of infancy: the experience of a large tertiary pediatric center in Israel. World J Pediatr 13(4):341–345

    CAS  PubMed  Google Scholar 

  • Pastuszczak M, Celińska-Löwenhoff M, Sulowicz J, Wojas-Pelc A, Musial J (2017) Clinical study on single-organ cutaneous small vessels vasculitis (SoCSVV). Medicine (Baltimore) 96(12):e6376

    Google Scholar 

  • Pillebout E, Thervet E, Hill G, Alberti C, Vanhille P, Nochy D (2002) Henoch-Schonlein purpura in adults: outcome and prognostic factors. J Am Soc Nephrol 13(5):1271–1278

    PubMed  Google Scholar 

  • Reed J, Wilkinson J (2000) Subcorneal pustular dermatosis. Clin Dermatol 18(3):301–313

    CAS  PubMed  Google Scholar 

  • Sangüeza OP, Pilcher B, Martin Sangüeza J (1997) Erythema elevatum diutinum: a clinicopathological study of eight cases. Am J Dermatopathol 19(3):214–222

    PubMed  Google Scholar 

  • Savino F, Lupica MM, Tarasco V, Locatelli E, Viola S, di Montezemolo LC, Coppo P (2013) Acute hemorrhagic edema of infancy: a troubling cutaneous presentation with a self-limiting course. Pediatr Dermatol 30(6):e149–e152

    PubMed  Google Scholar 

  • Sharma A, Dogra S, Sharma K (2015) Granulomatous vasculitis. Dermatol Clin 33(3):475–487

    CAS  PubMed  Google Scholar 

  • Sjowall C, Mandl T, Skattum L, Olsson M, Mohammad AJ (2018) Epidemiology of hypocomplementaemic urticarial vasculitis (anti-C1q vasculitis). Rheumatology (Oxford) 57(8):1400–1407

    Google Scholar 

  • Sunderkötter CH, Zelger B, Chen KR, Requena L, Piette W, Carlson JA, Dutz J, Lamprecht P, Mahr A, Aberer E, Werth VP, Wetter DA, Kawana S, Luqmani R, Frances C, Jorizzo J, Watts JR, Metze D, Caproni M, Alpsoy E, Callen JP, Fiorentino D, Merkel PA, Falk RJ, Jennette JC (2018) Nomenclature of cutaneous vasculitis: dermatologic addendum to the 2012 revised international Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheumatol 70(2):171–184

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Dermatology and Dermatopathology, University of Pittsburgh, Pittsburgh, PA, USA

    Viktoryia Kazlouskaya

  2. Department of Dermatology/Division of Dermatopathology, Geisinger Health System, Danville, PA, USA

    Jacqueline M. Junkins-Hopkins

Authors
  1. Viktoryia Kazlouskaya
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Jacqueline M. Junkins-Hopkins
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Jacqueline M. Junkins-Hopkins .

Editor information

Editors and Affiliations

  1. Department of Pathology, Univ of Rochester School of Med, Rochester, NY, USA

    Bruce Smoller

  2. Khorramshahr, Iran

    Nooshin Bagherani

Rights and permissions

Reprints and permissions

Copyright information

© 2020 Springer Nature Switzerland AG

About this entry

Check for updates. Verify currency and authenticity via CrossMark

Cite this entry

Kazlouskaya, V., Junkins-Hopkins, J.M. (2020). Vasculitis and Neutrophilic Dermatoses and Related Disorders. In: Smoller, B., Bagherani, N. (eds) Atlas of Dermatology, Dermatopathology and Venereology . Springer, Cham. https://doi.org/10.1007/978-3-319-45134-3_45-1

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-45134-3_45-1

  • Received:

  • Accepted:

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-45134-3

  • Online ISBN: 978-3-319-45134-3

  • eBook Packages: Springer Reference MedicineReference Module Medicine

Publish with us

Policies and ethics

Search

Navigation