Nomenclature of Cutaneous Vasculitis

Sunderkötter, Cord; Zelger, Bernhard; Chen, Ko Ron; Requena, Luis; Piette, Warren W.; Carlson, John A.; Dutz, Jan P.; Lamprecht, Peter; Mahr, Alfred; Aberer, Elisabeth; Werth, Victoria P.; Wetter, David A.; Kawana, Seiji; Luqmani, Raashid; Françès, Camille; Jorizzo, Joseph L.; Watts, Justin; Metze, Dieter; Caproni, Marzia; Alpsoy, Erkan; Callen, Jeffrey P.; Fiorentino, David; Merkel, Peter A.; Falk, Ronald J.; Jennette, J. Charles

doi:10.1002/art.40375

Cited by 227 publications

(24 citation statements)

References 67 publications

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“…Biopsies can differentiate between the different types of cutaneous vasculitis that can be seen in patients with SLE. 14 Despite these limitations, all diagnoses were made by…”

Section: Lupus Science and Medicinementioning

confidence: 99%

Cutaneous vasculitis in SLE

Goldman

Petri

2020

Lupus Sci Med

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ObjectivesWe determined the temporal association between clinical and serological disease manifestations and development of cutaneous small vessel vasculitis in a large prospective multiethnic cohort.MethodsPatients with SLE diagnosed according to the Systemic Lupus International Collaborating Clinics (SLICC) classification criteria or the revised classification criteria as defined by the American College of Rheumatology (ACR) were enrolled in the Hopkins Lupus Cohort. Cutaneous small vessel vasculitis was determined as a component of the Systemic Lupus Erythematosus Disease Activity Index. SLE-associated cutaneous small vessel vasculitis lesions were reported clinically. They presented as punctate lesions, palpable purpura, tender erythematous plaques or macules with or without necrosis. No histopathological diagnosis was pursued to confirm the diagnosis of vasculitis or to differentiate it from other causes of digital lesions in patients with SLE. Disease manifestations that preceded the first occurrence of cutaneous small vessel vasculitis lesions were analysed using Kaplan-Meier. Cox regression analysis was used to assess the relationship between baseline clinical and immunological manifestations and the development of cutaneous small vessel vasculitis. We adjusted for gender, race and age at SLE diagnosis.ResultsA total of 2580 patients were studied: 52.4% were Caucasian and 39.4% were African-American. The mean age of the cohort was 45.5±14.5 years. The mean years of cohort follow-up was 7.9±7.6. Cutaneous small vessel vasculitis was observed in 449 (17.3%). The mean time to cutaneous vasculitis after SLE diagnosis was 4.78 years (95% CI 3.96 to 5.60). At least 159 (35%) patients had recurrences of cutaneous vasculitis lesions. Discoid rash, Raynaud’s phenomenon, myositis, anaemia, Coombs’ positivity, leucopenia, anti-Smith and anti-RNP (Ribonucleoprotein) were significantly associated with the development of cutaneous vasculitis. The SLICC/ACR Damage Index score was higher in patients with cutaneous vasculitis compared with those without cutaneous vasculitis.ConclusionsCutaneous vasculitis is frequent (17.3%) and often recurrent (35%). African-Americans are at higher risk of developing cutaneous small vessel vasculitis than Caucasians. Clinical presentations such as myositis and haematological manifestations are predictors of cutaneous vasculitis development. The presence of cutaneous vasculitis is associated with increased organ damage.

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“…Biopsies can differentiate between the different types of cutaneous vasculitis that can be seen in patients with SLE. 14 Despite these limitations, all diagnoses were made by…”

Section: Lupus Science and Medicinementioning

confidence: 99%

Cutaneous vasculitis in SLE

Goldman

Petri

2020

Lupus Sci Med

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“…Cutaneous vasculitis is characterised by inflammation, damage and destruction of blood vessels of different sizes [176]. Cutaneous vasculitis is a quite common disease with an estimated incidence of 39.6/59.8 new cases/million persons/ year.…”

Section: Cutaneous Vasculitismentioning

confidence: 99%

“…Such cases are classified as paraneoplastic vasculitis. Most of these patients have an underling HM (90%), such as chronic myelomonocytic leukaemia, non-Hodgkin’s lymphoma, Hodgkin’s disease, B-cell chronic lymphatic leukaemia, and multiple myeloma [ 176 , 178 , 182 , 183 ]; whereas, paraneoplastic cutaneous vasculitis rarely occurs in patients with solid tumours (10% of cases), the most common being renal, breast and lung carcinomas [ 184 – 186 ].…”

Section: Miscellaneousmentioning

confidence: 99%

Immune-Mediated Dermatoses in Patients with Haematological Malignancies: A Comprehensive Review

et al. 2020

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Haematological malignancies induce important alterations of the immune system, which account for the high frequency of autoimmune complications observed in patients. Cutaneous immune-mediated diseases associated with haematological malignancies encompass a heterogeneous group of dermatoses, including, among others, neutrophilic and eosinophilic dermatoses, autoantibody-mediated skin diseases, vasculitis and granulomatous dermatoses. Some of these diseases, such as paraneoplastic pemphigus, are associated with an increased risk of death; others, such as eosinophilic dermatoses of haematological malignancies, run a benign clinical course but portend a significant negative impairment on a patient's quality of life. In rare cases, the skin eruption reflects immunological alterations associated with an unfavourable prognosis of the associated haematological disorder. Therapeutic management of immune-mediated skin diseases in patients with haematological malignancies is often challenging. Systemic corticosteroids and immunosuppressive drugs are considered frontline therapies but may considerably augment the risk of serious infections. Indeed, developing a specific targeted therapeutic approach is of crucial importance for this particularly fragile patient population. This review provides an up-to-date overview on the immune-mediated skin diseases most frequently encountered by patients with onco-haematological disorders, discussing new pathogenic advances and therapeutic options on the horizon.

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“…IgA vasculitis (IgAV) is characterized by inflammation of small blood vessels (histologically features of leukocytoclastic vasculitis) and IgA deposition primarily around postcapillary venules. The clinical hallmark of IgAV is palpable purpura with predilection for the lower limbs [1]. Systemic IgAV, formerly known as Henoch-Schönlein purpura (HSP), involves internal organs such as the kidneys (causing mesangioproliferative IgA nephritits), gut or joints in addition to the skin [2].…”

Section: Introductionmentioning

confidence: 99%

“…There is also a skin-limited form of IgAV, without systemic vasculitis, as recently defined in the interdisciplinary nomenclature of cutaneous vasculitides (D-CHCC 2012 [1], an addendum to the Chapel Hill Consensus Conference Nomenclature of Vasculitides CHCC 2012 [7]). Skin-limited IgAV does not usually progress into systemic IgAV and is more often seen by dermatologists than systemic IgAV, but there are no studies yet specifically addressing the respective incidence of skin-limited versus systemic IgAV.…”

Section: Introductionmentioning

confidence: 99%

Galactose-deficient IgA1 in skin and serum from patients with skin-limited and systemic IgA vasculitis

Neufeld

Molyneux

Pappelbaum

et al. 2019

Journal of the American Academy of Dermatology

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Background: IgA-vasculitis (IgAV) encompasses a systemic form involving kidneys, gut, skin or joints, and a skin-limited form. One characteristic feature of systemic IgAV is deposition of galactosedeficient IgA1 (GD-IgA1) in kidneys (as in IgA-nephropathy). The relevance of GD-IgA1 for cutaneous vasculitis is unknown. Objective: We investigated if GD-IgA1 is deposited perivascularly in systemic and also skin-limited IgAV, and if its serum levels differ between both forms. Methods: In a case control study, deposition of GD-IgA1 was analysed immunohistochemically by KM55-antibody in skin biopsies from 12 patients with skin-limited and 4 with systemic IgAV. GD-IgA1levels were compared by ELISA in sera from 15 patients each with skin-limited and systemic IgAV and from 11 healthy subjects. Results: All biopsies from systemic, but also from skin-limited IgAV revealed perivascular GD-IgA1deposition. The average GD-IgA1-level in serum was significantly higher in systemic than in skinlimited IgAV, despite overlap between both groups. Limitations: Although high GD-IgA1-levels may be predictive of systemic IgAV, patient numbers were too low to determine cutoff values for systemic versus skin-limited IgAV. Conclusion: While perivascular GD-IgA1-deposition is a prerequisite for systemic and skin-limited IgAV, high GD-IgA1-levels in some patients with systemic IgAV suggest a dose-dependent effect of GD-IgA1 in IgAV.

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Nomenclature of Cutaneous Vasculitis

Cited by 227 publications

References 67 publications

Cutaneous vasculitis in SLE

Cutaneous vasculitis in SLE

Immune-Mediated Dermatoses in Patients with Haematological Malignancies: A Comprehensive Review

Galactose-deficient IgA1 in skin and serum from patients with skin-limited and systemic IgA vasculitis

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