Phlebologie 2019; 48(04): 251-265
DOI: 10.1055/a-0847-6602
© Georg Thieme Verlag KG Stuttgart · New York

Vasculitis allergica - a non-IgE-mediated hypersensitivity syndrome

Article in several languages: deutsch | English
S. Lutze
Klinik und Poliklinik für Hautkrankheiten, Universitätsmedizin Greifswald
W. Konschake
Klinik und Poliklinik für Hautkrankheiten, Universitätsmedizin Greifswald
M. Ahmed
Klinik und Poliklinik für Hautkrankheiten, Universitätsmedizin Greifswald
A. Arnold
Klinik und Poliklinik für Hautkrankheiten, Universitätsmedizin Greifswald
T. Westphal
Klinik und Poliklinik für Hautkrankheiten, Universitätsmedizin Greifswald
H. Riebe
Klinik und Poliklinik für Hautkrankheiten, Universitätsmedizin Greifswald
G. Daeschlein
Klinik und Poliklinik für Hautkrankheiten, Universitätsmedizin Greifswald
M. Jünger
Klinik und Poliklinik für Hautkrankheiten, Universitätsmedizin Greifswald
› Author Affiliations
Further Information

Publication History

27 February 2018

29 January 2019

Publication Date:
10 May 2019 (online)


The diagnosis “Vasculitis allergica” suggests a classical allergic IgE-mediated genesis. However, this is not the case; rather, it is a highly complex mechanism that often prevents the antigen that actually triggers the antigen from being detected.

The current nomenclature for this dermatological disease pattern is cutaneous IgM or IgG immunocomplex vasculitis, which is leukocytoclastic vasculitis of the postcapillary venules [3]. This new classification discards the association associated with the original name with a typically IgE-mediated allergic disease.

Vasculitides are immunologically associated with immune complex diseases, the so-called type III reaction according to Coombs and Gell [14], [15], [16]. They are classified as “non-IgE-mediated allergic hypersensitivity syndrome” according to the underlying immunological process [14], [15], [16]. Many vasculitides are clinically first noticeable on the skin, e. g. by acute, rapidly progressive ulcerations, often associated with severe pain. They are therefore important in the clarification of the causes of venous leg ulcers, but here they rank among the rare diagnoses.

The long-standing term “leukocytoclastic vasculitis” for this disease no longer stands for a single disease pattern but rather for a histological pattern of a group of vasculitides, the so-called immune complex vasculitides [3]. Most of the vascular calibres are small vessel vasculitis, which can occur as single organ vasculitis (SOV) with sole manifestation on the skin or as a cutaneous partial symptom of another multi organ vasculitis (MOV), for example in the context of systemic lupus erythematosus [1]. Cutaneous IgM or IgG immunocomplex vasculitis shows the classic sudden, rapidly progressive course for vasculitis and is clinically characterized by a typical primary fluorescence, the palpable purpura, with high inflammation [2]. In the therapy concept, the first priority is to identify and eliminate the triggering agent [2], [42], [44]. Usually this type of vasculitis shows a predominantly residueless healing with low risk of recurrence, provided that the triggering agent is identified and eliminated [2], [41], [42], [44].

  • Literatur / References

  • 1 Jennette JC. et al. 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides, Arthritis & Rheumatism. 2013 65. 1-11
  • 2 Sunderkötter C. Vaskulitis und Vaskulopathien, Braun-Falco‘s Dermatologie. Venerologie und Allergologie 2018; 3: 1-44 DOI: 10.1007/978–3-662–49546–9_64–1
  • 3 Sunderkötter CH, Zelger B, Chen KR. et al. Nomenclature of Cutaneous Vasculitis Dermatologic Addendum to the 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides.
  • 4 Watts RA. Evolving concepts in classification of systemic vasculitis: where are we and what is the way forward?. International Journal of Rheumatic Diseases. 2018
  • 5 Stone JH. Kelley and Firestein’s Textbook of Rheumatology (Tenth Edition), 2017, Classification and Epidemiology of Systemic Vasculitis.
  • 6 Calabrese LH, Michel BA, Bloch DA. et al. The American College of Rheumatology 1990 criteria for the classification of hypersensitivity vasculitis. Arthritis Rheum. 1990
  • 7 Carlson JA, Ng BT, Chen KR. Cutaneous vasculitis update: diagnostic criteria, classification, epidemiology, etiology, pathogenesis, evaluation and prognosis. Am J Dermatopathol 2005; 27: 504-528
  • 8 Khetan P, Sethuraman G, Khaitan BK. et al. An aetiological & clinicopathological study on cutaneous vasculitis. Indian J Med Res 2012; 135: 107-113
  • 9 Sunderkötter C, Sindrilaru A. Classification of vasculitis. Europ J Dermatol 2006; 16 (02) 114-24
  • 10 Kawasaki Y. et al. Clinical and pathological features of children with Henoch-Schoenlein purpura nephritis: risk factors associated with poor prognosis. Clin Nephrol 2003; 60: 153-160
  • 11 Loricera J. et al. Single-organ cutaneous small-vessel vasculitis according to the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides: a study of 60 patients from a series of 766 cutaneous vasculitis cases. Rheumatology 2015; 54: 77-82
  • 12 Ratzinger G. et al. Das Vaskulitis-Rad-ein algorithmischer Ansatz für kutane Vaskulitiden. JDDG 2015; 1092-1118
  • 13 King TC. Elsevier’s Integrated Pathology, 2007, Inflammation, Inflammatory Mediators, and Immune-Mediated Disease.
  • 14 Birdsall HH. Mandell, Douglas, and Bennett’s Principles and Practice of Infectious Diseases (Eighth Edition), Adaptive Immunity. 2015
  • 15 Actor JK. Adaptive Immune Response and Hypersensitivity, in Elsevier’s Integrated Review Immunology and Microbiology (Second Edition). 2012
  • 16 Mak TW, Saunders ME. Allergy and Hypersensitivity. The Immune Response. 2006
  • 17 Lentsch AB, Ward PA. Regulation of inflammatory vascular damage. J Pathol. 2000
  • 18 Carlson JA. The histological assessment of cutaneous vasculitis. Histopathology 2010; 56: 3-23
  • 19 Stone JH, Nousari HC. ”Essential” cutaneous vasculitis: what every rheumatologist should know about vasculitis of the skin. Curr Opin Rheumatol 2001; 13: 23-34
  • 20 Jessop SJ. Cutaneous leucocytoclastic vasculitis: a clinical and aetiological study. Br J Rheumatol. 1995
  • 21 Sunderkötter C, Roth J, Bonsmann G. Leukozytoklastische Vaskulitis. Hautarzt 2004; 55 (08) 759-785
  • 22 Brouet. et al. Kryoglobulinämie. Am J Med 1974; 57: 775-788
  • 23 Staak JO, Glossmann JP. Diehl. et al. Hepatitis-C-Virus-assoziierte Kryoglobulinämie – Pathogenese, Diagnostik und Therapie. Medizinische Klinik October 2002; 97: 601-608
  • 24 Pischke S, Cornberg M, Manns MP. Hepatitis-assoziierte Kryoglobulinämie. Der Internist. 2008
  • 25 Davin JC. Henoch-Schonlein purpura nephritis. Pathophysiology, treatment, and future strategy. Clinical journal of the American Society of Nephrology 2011; 6: 679-689 DOI: 10.2215/CJN.06710810
  • 26 Blanco R, Martinez-Taboada VM, Rodriguez-Valverde V. et al. Cutaneous vasculitis in children and adults. Associated diseases and etiologic factors in 303 patients. Medicine (Baltimore). 1998
  • 27 Blanco R, Martinez-Taboada VM, Rodriguez-Valverde V. et al. Henoch-Schonlein purpura in adulthood and childhood: two different expressions of the same syndrome. Arthritis Rheum. 1997
  • 28 Calabrese LH, Duna GF. Drug-induced vasculitis. Curr Opin Rheumatol 1996; 8: 34-40
  • 29 Michel BA, Hunder GG, Bloch DA. Hypersensitivity vasculitis and Henoch- Schonlein purpura: a comparison between the 2 disorders. J Rheumatol. 1992
  • 30 Cervera R. Antiphospholipid sydrome. In: Thrombosis Research 2017; 151: 43-47 DOI: 10.2016/S0049–3848(17)30066-X
  • 31 Gómez-Puerta JA, Cervera R. Diagnosis and classification of the antiphospholipid syndrome. Journal of autoimmunity 2014; 48–49: 20-25 DOI:10.1016/j.jaut.2014.01.006
  • 32 Cervera R. Antiphospholipid syndrome. Thrombosis Research 2017; 151: 43-47 DOI: 10.1016/S0049–3848(17)30066-X
  • 33 Gómez-Puerta JA, Cervera R. Diagnosis and classification of the antiphospholipid syndrome. Journal of autoimmunity 2014; 48–49: 20-25 DOI: 10.1016/j.jaut.2014.01.006
  • 34 Miyakis S, Lockshin MD, Atsumi T. et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost 2006; 4: 295-306
  • 35 Kerk N, Goerge T. Livedoid vasculopathy – a thrombotic disease. VASA 2013; 42: 317-322
  • 36 Alavi A, Hafner J, Dutz JP. et al. Livedoid vasculopathy: an in-depth analysis using a modified Delphi approach. J Am Acad Dermatol 2013; 69: 1033-1042
  • 37 Jorizzo JL. Livedoid vasculopathy: what is it?. Arch Dermatol 1998; 134: 491-493
  • 38 Ahmed M, Lutze S, Herrmann A. et al. Livedovaskulopathie – eine Erkrankung seltener Blutgruppenmerkmale?. vasomed 2017; 29: 223-224
  • 39 Holder SM, Joy MS, Falk RJ. Cutaneous and systemic manifestations of drug-induced vasculitis. Ann Pharmacother. 2002
  • 40 Hengge UR. et al. Purpura fulminans. A fatal consequence of a widely used medication?. Hautarzt 2002; 53: 483-487
  • 41 Chen KR, Carlson JA. Clinical approach to cutaneous vasculitis. Am J Clin Dermatol 2008; 9: 71-92
  • 42 Carlson JA, Cavaliere LF, Grant-Kels JM. Cutaneous vasculitis: diagnosis and management. Clin Dermatol 2006; 24: 414-429
  • 43 Carlson JA, Chen KR. Cutaneous vasculitis update: small vessel neutrophilic vasculitis syndromes. Am J Dermatopathol 2006; 28: 486-506
  • 44 Sunderkötter C, Bonsmann G, Sindrilaru A. et al. Management of leukocytoclastic vasculitis. J Dermatolog Treat. 2005
  • 45 Averbeck M, Gebhardt C, Emmrich F. et al. Simon Immunologische Grundlagen der Allergien. JDDG 2007; 5: 1015-1028
  • 46 Ashfaq A, Marghoob MD, Malvehy J. et al. An Atlas of Dermoscopy, Second Edition (Englisch). Taschenbuch – 26. Juli 2012.
  • 47 Trautmann A, Kleine-Tebbe J. Allergologie in Klinik und Praxis: Allergene – Diagnostik – Therapie 25. Oktober 2017.
  • 48 Vázquez-López F, Fueyo A, Sánchez-Martín J. et al. Dermoscopy for the Screening of Common Urticaria and Urticaria Vasculitis. Arch Dermatol 2008; 144: 568 doi:10.1001/archderm.144.4.568
  • 49 Ashfaq AM, Alon S, Vazquez-Lopez F. et al. Dermoscopic Patterns of Purpuric Lesions. Arch Dermatol 2010; 146: 938 doi:10.1001/archdermatol.2010.162
  • 50 Chu-Sung Hu S, Chen GS, Lin CL. et al. Dermoscopic features of livedoid vasculopathy. Medicine (Baltimore) 2017; 96: e6284