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Cutaneous Manifestations of Medium- and Large-Vessel Vasculitis

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Clinical Reviews in Allergy & Immunology
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F. Chasset at Tenon Hospital
F. Chasset
  • Tenon Hospital
Camille Francès
Camille Francès
Camille Francès
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Abstract and Figures

Dermatologic manifestations are observed in almost all systemic vasculitides, even in large-and medium-vessel vasculitides, although such vessels are not found in the skin. Cutaneous manifestations may be related to a direct skin localization of the systemic vasculitis or a non-specific process associated with the vasculitis. According to the 2012 International Chapel Hill consensus, the two major variants of large-vessel vasculitides are Takayasu arteritis and giant-cell arteritis. In Europe and North America, acute inflammatory nodules or erythema nodosum-like lesions are the most commonly observed skin lesions with Takayasu arteritis. Medium-sized arteriole vasculitis of the dermis or subcutis but also septal or lobular panniculitis may be found during pathological examination. In Japan, widespread pyoderma gangrenosum-like lesions are more frequent. Cutaneous manifestations of giant-cell arteritis are rare; they are ischemic, linked to arterial occlusions, or non-ischemic, with various mechanisms. The two major medium-vessel vasculitides are Kawasaki disease and polyarteritis nodosa. Kawasaki disease is characterized by a mucocutaneous lymph node syndrome without skin vasculitis. Two subsets of polyarteritis nodosa with different skin manifestations are described, without transition from one to the other. In the systemic subset, the most frequent skin lesions are in the order of frequency purpura, livedo, and nodules. Cutaneous polyarteritis nodosa mainly features nodules, livedo racemosa, and ulcerations. Genetic screening and measurement of plasma levels of adenosine deaminase 2 should be considered for patients with uncommon systemic polyarteritis nodosa or early-onset cutaneous polyarteritis nodosa.
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Cutaneous Manifestations of Medium-
and Large-Vessel Vasculitis
Francois Chasset
1
&Camille Francès
1
Published online: 26 May 2017
#Springer Science+Business Media New York 2017
Clinic Rev Allerg Immunol (2017) 53:452–468
DOI 10.1007/s12016-017-8612-9
Abstract Dermatologic manifestations are observed in al-
most all systemic vasculitides, even in large-and medium-ves-
sel vasculitides, although such vessels are not found in the
skin. Cutaneous manifestations may be related to a direct skin
localization of the systemic vasculitis or a non-specific pro-
cess associated with the vasculitis. According to the 2012
International Chapel Hill consensus, the two major variants
of large-vessel vasculitides are Takayasu arteritis and giant-
cell arteritis. In Europe and North America, acute inflamma-
tory nodules or erythema nodosum-like lesions are the most
commonly observed skin lesions with Takayasu arteritis.
Medium-sized arteriole vasculitis of the dermis or subcutis
but also septal or lobular panniculitis may be found during
pathological examination. In Japan, widespread pyoderma
gangrenosum-like lesions are more frequent. Cutaneous man-
ifestations of giant-cell arteritis are rare; they are ischemic,
linked to arterial occlusions, or non-ischemic, with various
mechanisms. The two major medium-vessel vasculitides are
Kawasaki disease and polyarteritis nodosa. Kawasaki disease
is characterized by a mucocutaneous lymph node syndrome
without skin vasculitis. Two subsets of polyarteritis nodosa
with different skin manifestations are described, without tran-
sition from one to the other. In the systemic subset, the most
frequent skin lesions are in the order of frequency purpura,
livedo, and nodules. Cutaneous polyarteritis nodosa mainly
features nodules, livedo racemosa, and ulcerations. Genetic
screening and measurement of plasma levels of adenosine
deaminase 2 should be considered for patients with uncom-
mon systemic polyarteritis nodosa or early-onset cutaneous
polyarteritis nodosa.
Keywords Takayasu arteritis .Giant-cell arteritis .
Polyarteritis nodosa .Kawasaki disease
Abbreviations
ACR American College of Rheumatology
ADA2 Adenosine deaminase 2
ANCA Anti-neutrophil cytoplasmic antibodies
CECR1 Cat eye syndrome chromosome region candidate 1
gene
CHCC Chapel Hill Consensus Conference
EMA European Medicines Agency
EULAR European League Against Rheumatism
FFS Five-factor score
GCA Giant-cell arteritis
G6PD Glucose-6-phosphate dehydrogenase
HBV Human hepatitis B virus
KD Kawasaki disease
LVV Large-vessel vasculitis
LVVs Large-vessel vasculitides
MLA Macular lymphocytic arteritis
MVV Medium-vessel vasculitis
MVVs Medium-vessel vasculitides
PAN Polyarteritis nodosa
SoJIA Systemic onset juvenile idiopathic arthritis
SOV Single-organ vasculitis
TAK Takayasu arteritis
TSS Toxic shock syndrome
*Camille Francès
camille.frances@aphp.fr
1
Université Pierre et Marie Curie-Paris VI, Assistance
Publique-Hôpitaux de Paris, Service de Dermatologie-Allergologie,
Hôpital Tenon, 4 rue de la Chine, 75020 Paris, France
Content courtesy of Springer Nature, terms of use apply. Rights reserved.
... The prevalence of the latter is estimated to be around 2.8 to 28% of TA patients [3][4][5][6][7] . From a clinical and histological point of view, cutaneous manifestations can be divided into specific for TA -acute inflammatory nodules, erythema nodosum-like subcutaneous nodules, erythema induratum, pyoderma gangrenosum-like ulcers, livedo reticularis, and purpuric and necrotic lesions -and nonspecific -urticaria-like lesions, erythematous macules and papules, and eczematiform lesions, among others 3,[8][9][10][11] . More than one type of lesion may be present in the same patient 11 . ...
... The first skin manifestations in TA were described in 1985 by Mousa et al. 12 , who associated the nodular skin lesions with the systemic vasculitis in a patient with TA. Overall, dermatologic manifestations in patients with TA may result from the occlusion of large vessels -presenting as Raynaud's phenomenon and digital gangrene -or from the inflammation of small cutaneous vessels 3,9 . The latter may manifest as erythema nodosum, ulcerated nodules, and pyoderma gangrenosum-like lesions, among others 3,[8][9][10][11] . ...
... Overall, dermatologic manifestations in patients with TA may result from the occlusion of large vessels -presenting as Raynaud's phenomenon and digital gangrene -or from the inflammation of small cutaneous vessels 3,9 . The latter may manifest as erythema nodosum, ulcerated nodules, and pyoderma gangrenosum-like lesions, among others 3,[8][9][10][11] . In North America and Europe, acute inflammatory nodules and erythema nodosum are the most prevalent skin manifestations 3 , while pyoderma gangrenosum has been described more frequently in the TA Japanese population and predominantly affects the upper arms 9 . ...
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... Takayasu arteritis has been reported to present with skin lesions, including erythema nodosum-like lesions in Europe and North America, and pyoderma gangrenosumlike lesions in Japan [41]. ...
Medium and Large Vessel Vasculitis, Clinical Review for the Inpatient Dermatologist
Article
Full-text available
  • Jan 2025
Purpose of Review Medium and, rarely, large vessel vasculitis may affect the skin, and dermatologists can be instrumental in making a timely diagnosis. The purpose of this review is to present a general overview of medium and large vessel vasculitis presenting in the skin, with a focus on the latest research. Recent Findings Newly published studies on cutaneous polyarteritis nodosa have highlighted novel associations, such as VEXAS and DADA2. Updated classification criteria for ANCA associated vasculitis (AAV) were recently published by the American College of Rheumatology. Avacopan, mepolizumab, and benralizumab are exciting new therapies for AAV. Summary Much progress has been made towards the goal of a structured, evidence-based approach to cutaneous vasculitis. While work remains to be done, standardization of disease classifications and phase 3 clinical trials have paved the way for novel therapies, particularly in ANCA associated vasculitis. This review will provide an overview of medium and large vessel vasculitis for the practicing dermatologist.
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... La AT en la piel puede causar púrpura, livedo reticularis, lesiones subcutáneas similares al eritema nodoso, eritema indurado, nódulos inflamatorios, nódulos necróticos y/o ulcerados, gangrena digital, úlceras similares al pioderma gangrenoso, fenómeno de Raynaud, urticaria, angioedema y eritema multiforme. 15,16 Es necesario ampliar estudios en nuestra paciente para aclarar la naturaleza de sus lesiones dérmicas. ...
Neumonía organizativa como manifestación inicial de arteritis de Takayasu: Reporte de un caso
Article
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  • Jul 2023
La arteritis de Takayasu (AT) es una enfermedad inflamatoria rara que afecta a grandes vasos, como la aorta y sus ramas. Los síntomas iniciales son variados y pueden incluir mareos, fiebre, lesiones cutáneas y mialgias. El diagnóstico se realiza con criterios clínicos, pruebas de imagen, laboratorio e histopatología. Las manifestacionespulmonares constituyen formas de presentación muy infrecuentes e incluyen neumonía organizativa, efusión pleural e hipertensión pulmonar. Presentamos el caso de una mujer asmática joven, con historia de neumonitisrecurrente y disnea progresiva, asociados a compromiso inflamatorio de grandes vasos por arteritis de Takayasu.
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Livedos
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  • Oct 2019
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Cutaneous polyarteritis nodosa and pulmonary arterial hypertension: An unexpected liaison. A case report
Article
  • Dec 2023
Background Cutaneous polyarteritis nodosa (cPAN) is a form of medium-sized vessel necrotizing vasculitis. It is a rare, skin-limited variant of polyarteritis nodosa, characterized by dermal and subcutaneous tissue involvement. The most common findings in cPAN include digital gangrene, livedo reticularis, and tender subcutaneous nodules. However, while limited to the skin, cPAN results in significant morbidity and mortality due to the accompanying skin ischemia and necrosis, such that patients are vulnerable to superinfection. Here, we describe a unique presentation of cPAN associated with pulmonary arterial hypertension (PAH). Methods A 78-year-old female presented with digital ischemia and leg ulcers associated with PAH. Skin biopsy showed necrotizing fibrinoid necrosis of the small- and middle-sized vessels of the dermis. A diagnosis of cPAN and PAH was made. The patient was treated with glucocorticoids, vasodilators, and cyclophosphamide. Results She died due to severe sepsis complications. Conclusion To date, this is the first case report describing the association between cPAN and PAH. In this case, PAH is a complication of the cutaneous vasculitides suggesting that vasculopathy could play a role in the pathophysiology of PAH. However, the underlying pathophysiological mechanisms still have to be firmly established.
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Sarcoidal Granuloma Annulare-Like Dermatitis and Vasculitis With Granulomatous Features: An Atypical Case of Giant Cell Arteritis
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  • Sep 2023
Giant cell arteritis (GCA) is a diagnosis that clinicians should not miss because of the accompanying risk of irreversible vision loss. GCA can present without the classic symptoms of headache and temporal artery tenderness, which may lead to a delay in diagnosis. Cutaneous findings, although rare, have been associated with GCA. Accordingly, it is imperative to be aware of the broad clinical and histological presentations of GCA, including the cutaneous findings, because they may prove to be harbingers of impending disease. We present a unique case of GCA where 2 distinct cutaneous morphologies, sarcoidal granuloma annulare-like dermatitis and leukocytoclastic vasculitis with granulomatous features, presented simultaneously before the classic symptoms of headache and unilateral vision loss.
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An Unusual Association of Chronic Recurrent Multifocal Osteomyelitis, Pyoderma Gangrenosum, and Takayasu Arteritis
Article
Full-text available
  • Jan 2017
To the Editor: Chronic recurrent multifocal osteomyelitis (CRMO) is hypothesized to be an autoimmune disorder because of its association with multiple autoimmune diseases, including inflammatory bowel disease, psoriasis, acne, pustulosis, Sweet syndrome, dyserythropoietic anemia, pyoderma gangrenosum (PG), sclerosing cholangitis, inflammatory arthritis, Still disease, Takayasu arteritis (TA), Ollier disease, and dermatomyositis1,2. PG association with TA is rare; however, Ujiie, et al showed that PG is associated with TA in 33% of patients3. Occurrence of all 3 conditions together (CRMO, PG, and TA) is very rarely reported. A 10-year-old girl born to nonconsanguineous parents presented with a history of intermittent swelling over the right side of the mandible, associated with pain and claudication pain over legs and back with breathlessness on exertion for the past 2 years. She also developed ulcerative cauliflower-like skin lesions over the dorsum of left foot over the past 2 months (Figure 1). On examination, her pulses were absent … Address correspondence to Dr. S. Kumar, Christian Medical College, Department of Pediatrics, Christian Medical College, Vellore, Tamil Nadu 632004, India. E-mail: sathishkumar{at}cmcvellore.ac.in
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Pyoderma Gangrenosum and Erythema Nodosum Revealing Takayasu’s Arteritis
Article
Full-text available
  • Dec 2016
We report a Caucasian female who presented with simultaneous erythema nodosum and pyoderma gangrenosum due to underlying Takayasu’s arteritis. Takayasu’s arteritis is a chronic large vessel vasculitis of unknown cause. The disease has a worldwide distribution but is most commonly seen in Asian populations. There is a strong predilection for young females. The clinical presentation is variable, but mostly derives from stenosis or occlusion of affected arteries, resulting in claudication and ischemia. Skin manifestations are observed in up to 28% of patients with Takayasu’s arteritis, with erythema nodosum reported more frequently in Caucasians. Pyoderma gangrenosum is more common in Asian patients. This report demonstrates the importance to exclude Takayasu’s arteritis in patients with such skin lesions.
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Kawasaki disease for dermatologists
Article
Full-text available
  • Nov 2016
Kawasaki disease (KD) is a systemic vasculitis that mostly affects children below the age of 5. The vasculitis involves arteries of medium size, especially the coronaries. Various etiologies have been proposed including association with micro-organisms, bacterial superantigens, and genetic factors, however, the exact cause remains unknown. There is no specific laboratory test for KD. Diagnosis is clinical and depends upon the presence of fever for ≥5 days and 4 or more of five principal features, viz. polymorphous exanthem, extremity changes, mucosal changes involving the lips and oral cavity, bilateral bulbar conjunctival injection, and unilateral cervical lymphadenopathy. The term “incomplete KD” refers to the presence of fever and less than four principal clinical features. Recognition of this group of patients is important because it is usually seen in infants and risk of coronary abnormalities is increased probably because of delays in diagnosis. However, what appears to be “incomplete” at a given point of time may not actually be so because some of the features may have already subsided and others may evolve over time. Hence, a detailed dermatological examination is warranted in all cases, especially in incomplete KD, to ensure timely diagnosis. Although KD is a self-limiting disease in most patients, coronary artery abnormalities (CAAs) including coronary dilatations and aneurysms may develop in up to 25% of untreated patients. CAAs are the most common cause of morbidity and mortality in patients with KD. Treatment is aimed at reducing inflammation and consists of intravenous immunoglobulin (IVIG) along with aspirin. Despite treatment, some patients may still develop CAAs, and hence, long-term follow up is of utmost importance.
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The Clinical Diagnosis and Management of Kawasaki Disease: a Review and Update
Article
Full-text available
  • Sep 2016
  • Curr Infect Dis Rep
Kawasaki disease is an acute, self-limited vasculitis of childhood and has become the leading cause of acquired pediatric heart disease in the USA. Prompt treatment is essential in reducing cardiac-related morbidity and mortality. The underlying etiology remains unknown. The disease itself may be the characteristic manifestation of a common pathway of immune-mediated vascular inflammation in susceptible hosts. The characteristic clinical features of fever for at least 5 days with bilateral nonpurulent conjunctivitis, rash, changes in lips and oral cavity, changes in peripheral extremities, and cervical lymphadenopathy remain the mainstay of diagnosis. Supplementary laboratory criteria can aid in the diagnosis, particularly in cases of incomplete clinical presentation. Diagnosis of Kawasaki disease can be challenging as the clinical presentation can be mistaken for a variety of other pediatric illnesses. Standard of care consists of intravenous immune globulin and aspirin. Corticosteroids, infliximab, and cyclosporine A have been used as adjunct therapy for Kawasaki disease refractory to initial treatment. There is ongoing research into the use of these agents in the initial therapy of Kawasaki disease.
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A randomized, double-blind trial of abatacept (CTLA4-ig) for the treatment of takayasu's arteritis: Abatacept for the treatment of Takayasu's arteritis
Article
  • Jan 2017
Objective: To compare the efficacy of abatacept to placebo for the treatment of Takayasu's arteritis (TAK). Methods: In this multicenter trial, patients with newly-diagnosed or relapsing TAK were treated with abatacept 10 mg/kg IV on days 1, 15, 29, week 8, together with prednisone. At week 12, patients in remission underwent a double-blinded randomization to continue monthly abatacept or switch to placebo. Patients in both study arms received a standardized prednisone taper, reaching 20 mg daily at week 12 with discontinuation of prednisone at week 28 and remained on their randomized assignment until meeting criteria for early termination or until 12 months after enrollment of the last patient. The primary endpoint was duration of remission (relapse-free survival). Results: Thirty-four eligible patients with TAK were enrolled and treated with prednisone and abatacept; 26 reached the week 12 randomization and underwent a blinded randomization to abatacept or placebo. The relapse-free survival at 12 months was 22% for those receiving abatacept and 40% for those receiving placebo (p= 0.853). Treatment with abatacept in patients with TAK enrolled in this study was not associated with a longer median duration of remission (abatacept 5.5 months, placebo 5.7 months). There was no difference in the frequency or severity of adverse events between treatment arms, including infection. Conclusions: In patients with TAK the addition of abatacept to a treatment regimen with prednisone did not reduce the risk of relapse. This article is protected by copyright. All rights reserved.
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Gastrointestinal aspects of vasculitides
Article
  • Nov 2016
Systemic vasculitides are caused by inflammation of blood vessels and can affect any organ and any part of the gastrointestinal tract, hepatic and biliary system, as well as the pancreas. These disorders can cause a wide array of gastrointestinal manifestations, from asymptomatic elevated transaminase levels and mild abdominal pain to potentially life-threatening bowel perforations and peritonitis. A diagnosis based solely on gastrointestinal symptoms is challenging as these manifestations are not specific. Conversely, diagnostic and therapeutic delays can be rapidly detrimental. In this article, we review the epidemiology, characteristics and management of the main gastrointestinal manifestations of systemic vasculitides, including polyarteritis nodosa and antineutrophil cytoplasm antibody-associated vasculitides, as well as isolated vasculitides limited to the gastrointestinal tract.
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Absence of mycobacterial DNA in peripheral blood and artery specimens in patients with Takayasu arteritis
Article
  • Sep 2016
The objective of this study was to demonstrate the presence of mycobacterial nucleic acid sequences in peripheral blood and arteries from patients with Takayasu arteritis (TA). Polymerase chain reaction was performed to detect mycobacterial DNA from three different nucleic acid sequences including the insertion sequence (IS) 6110, the 65-kDa heat shock protein gene (HSP65), and the 16S ribosomal RNA (rRNA) gene in peripheral blood from 32 TA patients and in arterial specimens from 10 TA patients. Twenty-eight HIV-negative patients with pulmonary tuberculosis prior to therapy were tested for IS6110 in peripheral blood as positive controls, and 24 blood donors were evaluated as healthy controls (HC). All TA patients were negative for the insertion sequence IS6110 and for HSP65 and 16S rRNA genes in blood samples and in arterial specimens. IS6110 sequence was found in peripheral blood from 22 (78.5 %) patients with pulmonary tuberculosis but not in HC. In conclusion, the strategy of mycobacterial-specific nucleic acid amplification in the peripheral blood and arterial specimens of TA patients was unable to lend support to the association between TA and tuberculosis long suggested in the literature.
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Incidence, Prevalence, and Survival of Biopsy-Proven Giant Cell Arteritis in Northern Italy During a 26-Year Period: GCA Epidemiology in Italy
Article
  • May 2016
Objectives: To investigate the epidemiology and mortality in patients with biopsy-proven giant cell arteritis (GCA) in Northern Italy. Methods: All patients with incident temporal artery biopsy-positive GCA diagnosed between 1986 and 2012 living in the Reggio Emilia area were identified using pathology register and by reviewing all histopathological specimens. For each patient, we identified one comparison subject from the same geographic area matched for age and gender. Mortality rates and specific causes of death were reported. Results: There were 285 incident cases of biopsy-proven GCA (210 women) during the 26-year study period. The overall age- and sex-adjusted incidence per 100,000 persons aged 50 years or older was 5.8 (95%CI: 5.1 to 6.5). Incidence was significantly higher in women (7.8, 95%CI: 6.7 to 8.9) than in men (3.3, 95%CI: 2.6 to 4.1) (p<0.0001). Annual age- and sex-adjusted incidence rates significantly increased by 15.9% per 3 years from 1986 to 2000, then significantly fell by -4.8% per 3 years from 2001-2012. The prevalence of GCA on December 31, 2012 was 87.9 (95%CI: 75.8 to 101.4). No significant differences in the mortality rates were observed between GCA patients (4.9 per 100 person-year, 95%CI: 4.1 to 5.8) and non-GCA subjects (5.6, 95%CI: 4.7 to 6.6). No significant differences in causes of death were observed comparing GCA patients to non-GCA subjects. Conclusions: This large population-based study of biopsy-proven GCA confirmed the lower incidence of GCA in Mediterranean countries and did not observe any increased mortality risk. This article is protected by copyright. All rights reserved.
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