Cutaneous vasculitis

Vasculitis is a term that refers to damage and inflammation of the walls of blood vessels of any size. The classification of types of cutaneous vasculitis continues to be a challenge, probably because of our lack of understanding of the etiology and pathogenesis of this condition. Changes in the vessel wall will be visible on microscopy and will enable the different clinical forms to be distinguished according to the caliber of affected vessels, the type of cell that predominates in the inflammatory infiltrate, or the presence of such key findings as extravascular granulomas. Skin manifestations (macules, papules, nodules, livedo reticularis, etc) correlate with the size of the vessel affected. The prognosis in cases of vasculitis with skin involvement will be determined by the presence or absence of extracutaneous disease. Systemic vasculitis shows a predilection for certain organs, such as the kidney or lung. The introduction of immunosuppressant drug treatments has led to evident improvement in survival rates for patients with vasculitis. This review covers practical aspects of the pathophysiology, histopathology, treatment, and differential diagnosis of the main clinical presentations of vasculitis with cutaneous involvement.     

Cutaneous vasculitis update: neutrophilic muscular vessel and eosinophilic, granulomatous, and lymphocytic vasculitis syndromes

Most biopsies of cutaneous vasculitis will exhibit a small vessel neutrophilic vasculitis [leukocytoclastic vasculitis (LCV)] that is associated with immune complexes on direct immunofluorescence examination or, less commonly, antineutrophilic cytoplasmic antibodies (ANCA) by indirect immunofluorescence testing. Is in uncommon for skin biopsy to reveal solely a neutrophilic arteritis signifying the presence of cutaneous polyarteritis nodosa or, if accompanied by significant lobular panniculitis, nodular vasculitis/erythema induratum. In other cases, cutaneous vascular damage (fibrinoid necrosis, muscular vessel wall disruption, or endarteritis obliterans) will be mediated by a nonneutrophilic inflammatory infiltrate. Eosinophilic vasculitis can be a primary (idiopathic) process that overlaps with hypereosinophilic syndrome, or it can be a secondary vasculitis associated with connective tissue disease or parasite infestation. Authentic cutaneous granulomatous vasculitis (versus vasculitis with extravascular granulomas) can represent a cutaneous manifestation of giant cell arteritis, an eruption secondary to systemic disease such as Crohn's disease or sarcoidosis, or a localized disorder, often a post-herpes zoster (HZ) phenomenon. Lymphocytic vasculitis is a histologic reaction pattern that correlates with broad clinical differential diagnosis, which includes connective tissue disease - mostly systemic lupus erythematosus (SLE), endothelial infection by Rickettsia and viruses, idiopathic lichenoid dermatoses such as perniosis or ulcerative necrotic Mucha-Habermann disease, and angiocentric cutaneous T-cell lymphomas. Skin biopsy extending into the subcutis, identifying the dominant inflammatory cell and caliber of vessels affected, extravascular histologic clues such as presence of lichenoid dermatitis or panniculitis, and correlation with clinical data allows for accurate diagnosis of these uncommon vasculitic entities.     

Cutaneous vasculitis

Vasculitis can range in severity from a self-limited single-organ disorder to a life-threatening disease with the prospect of multiple-organ failure. This condition presents many challenges to the physician, including classification and diagnosis, appropriate laboratory workup, treatment, and the need for careful follow-up. The physician must not only be able to recognize vasculitis but also be able to provide a specific diagnosis (if possible) as well as recognize and treat any underlying etiologic condition. Most diagnostic criteria are based on the size of vessel involvement, which often correlates with specific dermatologic findings. This may allow the dermatologist to provide an initial diagnosis and direct the medical evaluation. This article reviews the classification and diagnosis of cutaneous vasculitic syndromes and current treatment options; it also presents a comprehensive approach to diagnosing and treating the patient with suspected cutaneous vasculitis. (J Am Acad Dermatol 2003;48:311-40.) LEARNING OBJECTIVE: At the completion of this learning activity, participants should be familiar with the classification and clinical features of the various forms of cutaneous vasculitis. They should also have a rational approach to diagnosing and treating a patient with vasculitis.     

Cutaneous vasculitis update: small vessel neutrophilic vasculitis syndromes

A broad and diverse spectrum of vasculitic syndromes exists. These syndromes affect the skin with varying levels of associated systemic manifestations, running the gamut from a self-limited, localized, cutaneous phenomenon to rapidly progressive, multiorgan disease. The majority of cases of cutaneous vasculitis will show a neutrophilic small vessel vasculitis that can be either a primary (idiopathic) disorder (eg, cutaneous leukocytoclastic angiitis) or a secondary disorder that is associated with drugs, infection (eg, streptococcal infection, viral hepatitis), or underlying disease (eg, connective tissue disease, malignancy). Biopsy is the gold standard for the diagnosis of cutaneous vasculitis and also necessary for the detection of cutaneous vascular immune complexes by direct immunofluorescence. Based on the type of vessel disrupted by inflammation (small and/or muscular), the distribution of vasculitis in the dermis and subcutis, and predominate inflammatory cell-type mediating vessel wall damage, a list of relevant differential diagnoses can be generated. This histologic information coupled with extravascular findings such as tissue eosinophilia, tissue neutrophilia, and/or granulomas, plus pathophysiologic markers such as direct immunofluorescent examination for immune complexes and serologic evaluation for antineutrophil cytoplasmic antibodies allows for more accurate diagnosis of specific vasculitic entities. Herein, we review both primary and secondary vasculitic syndromes that affect the skin and show a small vessel neutrophilic mediated vasculitis.     

Update on vasculitis: an overview and dermatological clues for clinical and histopathological diagnosis – part I

The term vasculitis refers to the inflammation of vessel walls. It may range in severity from a self-limited disorder in one single organ to a life-threatening disease due to multiple organ failure. It has many causes, although they result in only a few histological patterns of vascular inflammation. Vessels of any type and in any organ can be affected, a fact that results in a broad variety of signs and symptoms. Different vasculitides with indistinguishable clinical presentations have quite different prognosis and treatments. This condition presents many challenges to physicians in terms of classification, diagnosis, appropriate laboratory workup, and treatment. Moreover, it compels a careful follow-up. This article reviews the Chapel-Hill 2012 classification, etiology, recent insights in pathophysiology, some important dermatological clues for the diagnosis and summarizes treatment of some of these complex vasculitis syndromes.     

Vasculitic wheel – an algorithmic approach to cutaneous vasculitides

Background: Previous classifications of vasculitides suffer from several defects. First, classifications may follow different principles including clinicopathologic findings, etiology, pathogenesis, prognosis, or therapeutic options. Second, authors fail to distinguish between vasculitis and coagulopathy. Third, vasculitides are systemic diseases. Organ‐specific variations make morphologic findings difficult to compare. Fourth, subtle changes are recognized in the skin, but may be asymptomatic in other organs. Our aim was to use the skin and subcutis as a model and the clinicopathologic correlation as the basic process for classification. Methods and Results: We use an algorithmic approach with pattern analysis, which allows for consistent reporting of microscopic findings. We first differentiate between small and medium vessel vasculitis. In the second step, we differentiate the subtypes of small (capillaries versus postcapillary venules) and medium‐sized (arterioles/arteries versus veins) vessels. In the final step, we differentiate, according to the predominant cell type, into leukocytoclastic and/or granulomatous vasculitis. Conclusions: Starting from leukocytoclastic vasculitis as a central reaction pattern of cutaneous small/medium vessel vasculitides, its relations or variations may be arranged around it like spokes of a wheel around the hub. This may help establish some basic order in this rather complex realm of cutaneous vasculitides, leading to a better understanding in a complicated field.     

New algorithm (KAWAKAMI algorithm) to diagnose primary cutaneous vasculitis

Palpable purpura tends to indicate involvement of small vessel vasculitis in the upper dermis. Livedo racemosa, nodular lesion and skin ulceration are indicative of involvement of small to medium-sized vessel vasculitis in the lower dermis to subcutaneous fat. We set out to establish a new algorithm (KAWAKAMI algorithm) for primary cutaneous vasculitis based on the Chapel Hill Consensus Conference classification and our research results, and apply to the diagnosis. The first step is to measure serum antineutrophil cytoplasmic antibodies (ANCA) levels. If myeloperoxidase-ANCA is positive, Churg–Strauss syndrome or microscopic polyangiitis can be suspected, and if the patient is positive for proteinase 3-ANCA, Wegener's granulomatosis is most likely. Next, if cryoglobulin is positive, cryoglobulinemic vasculitis should be suspected. Third, if direct immunofluorescence of the skin biopsy specimen reveals immunoglobulin A deposition within the affected vessels, Henoch–Schönlein purpura is indicated. Finally, the presence of anti-phosphatidylserine–prothrombin complex antibodies and/or lupus anticoagulant and histopathological necrotizing vasculitis in the upper to middle dermis (leukocytoclastic vasculitis) indicates cutaneous leukocytoclastic angiitis, whereas if necrotizing vasculitis exists in the lower dermis and/or is associated with the subcutaneous fat, cutaneous polyarteritis nodosa is indicated. The KAWAKAMI algorithm may allow us to refine our earlier diagnostic strategies and allow for efficacious treatment of primary cutaneous vasculitis. In cutaneous polyarteritis nodosa, warfarin or clopidogrel therapies should be administrated, and in cases that have associated active inflammatory lesions, corticosteroids or mizoribine (mycophenolate mofetil) therapy should be added. We further propose prophylactic treatment of renal complications in patients with Henoch–Schönlein purpura.     

Diagnostic, prognostic and pathogenic value of the direct immunofluorescence test in cutaneous leukocytoclastic vasculitis

BACKGROUND: No precise studies have been performed on cutaneous leukocytoclastic vasculitis (LV) to establish whether it is better to obtain a skin biopsy from lesional or from perilesional skin for direct immunofluorescence (DIF). There is no agreement on the immunoglobulins most frequently detected and the value of DIF for the classification of cutaneous vasculitis. METHODS: A prospective study of DIF in lesional and perilesional skin was performed in 50 leukocytoclastic vasculitis patients and 15 nonvasculitis patients. RESULTS: We detected a higher level of positivity in involved skin than in uninvolved skin for IgG, IgA, IgM, C3 and fibrinogen but not for C1q. In vasculitic patients, IgA was the immunoglobulin most frequently detected in lesional (82%) and perilesional skin (68%), followed by IgM (56 and 34%, respectively) and IgG (20 and 8%, respectively). Only IgA deposits were associated with the diagnosis of vasculitis, with a sensitivity of 82% in lesional and 68% in perilesional skin, and with a specificity of 73 and 66.7%, respectively. The presence of IgA in lesional skin was associated with renal involvement but there was no association with severity. The presence of IgG or IgM, or the absence of IgA in perilesional skin was related to the presence of cryoglobulins. The absence of IgA in lesional and perilesional skin was also related to hepatitis C virus infection. CONCLUSIONS: DIF findings in involved skin are more closely related to the diagnosis of vasculitis and can give more information about overall renal involvement than findings in uninvolved skin. However, findings in uninvolved skin are more closely related to the pathogenic factors that trigger the development of vasculitis.     

The spectrum of cutaneous granulomatous vasculitis: histopathologic report of eight cases with clinical correlation

Cutaneous granulomatous vasculitis is an uncommon histopathologic finding that has been associated with lymphoproliferative disorders, systemic vasculitis, autoimmune inflammatory diseases, and infection. To define further the concept of cutaneous granulomatous vasculitis and to emphasize its clinical importance, we reviewed biopsy material from 8 patients seen from 1985 through 1992. All biopsies showed evidence of blood vessel damage with fibrinoid change or hemorrhage (or both) and granulomatous inflammation in and around vessel walls. Special stains for microorganisms were negative in all cases. Associated medical disorders included neuropathy (2 patients), sarcoid-like disease (2), systemic vasculitis (1), lymphoma and suspected lymphoma (1 each), and associated herpes simplex virus (1). T-cell gene-rearrangement studies were negative in a patient with suspected lymphoma. Granulomatous cutaneous vasculitis is most commonly associated with lymphoma and systemic vasculitis. In selected cases, infection should be considered as an underlying cause.     

Livedo reticularis de las piernas: Metodología de diagnóstico y tratamiento

The term livedo reticularis refers to a reddish-violet reticular discoloration of the skin that mainly affects the limbs. It is caused by an interruption of blood flow in the dermal arteries, either due to spasm, inflammation, or vascular obstruction, and is associated with diseases of varying etiology and severity. To establish the cause of livedo reticularis, it is essential to determine its course (chronic, acute, or fulminant), the presence of other cutaneous signs such as nodules, retiform purpura or necrosis, and the possible association of general symptoms or laboratory findings that suggest a particular systemic process. The aim of this review is to describe the diagnosis and treatment of the disease.     

Sweet syndrome with immune complex vasculitis in a child

We report on a rare case of Sweet's syndrome (SS) in a 7-year-old boy showing typical symptoms such as high fever, neutrophil leucocytosis in peripheral blood, elevated blood sedimentation rate, and arthralgia. The cutaneous lesions were painful erythematous, raised plaques on both limbs and the face. Histology confirmed the clinical diagnosis, including remarkable signs of cutaneous small vessel vasculitis, which was verified by direct immunofluorescence for IgM and complement (C3) in vessel walls as well as by the presence of circulating immune complexes in the blood. By means of monoclonal antibodies, T-lymphocytes and macrophages could be identified in the perivascular infiltrates in the delayed phase of SS. According to these immunological findings, we discuss the pathomechanism and the therapeutical benefit of inhibitors of cyclooxygenase.     

Initial cutaneous manifestations associated with histopathological leukocytoclastic vasculitis in two patients with antiphospholipid antibody syndrome

Antiphospholipid antibody syndrome (APS) is a multisystem disorder associated with a variety of circulating autoantibodies that target different phospholipid protein complexes. APS is sometimes lethal as a result of severe sequelae, which may be primary or secondary to the underlying disease. We report two women who presented histopathologically with leukocytoclastic vasculitis as the first cutaneous manifestation and were subsequently diagnosed with APS associated with systemic lupus erythematosus (SLE). Patient 1 presented with widespread cutaneous necrosis (WCN) with rapidly spreading pain down the lower extremities. Skin biopsy specimens from her leg purpura and WCN revealed perivascular infiltrates with neutrophils consistent with leukocytoclastic vasculitis and thromboses of small-sized dermal vessels. Patient 2 exhibited livedo reticularis, painful cutaneous nodules with necrosis, ulcer, and erythematous macules on her lower extremities, shoulder, and face. Skin biopsies of her right knee showed intravascular thrombosis of small dermal vessels and infiltration of perivascular tissues with necrotizing granulomatous vasculitis in the dermis. We found that these various cutaneous manifestations with leukocytoclastic vasculitis were present at an early stage of APS. Although progression to leukocytoclastic vasculitis in patients with APS is uncommon, our data suggest that the association between microvascular occlusions and cutaneous vessel vasculitis has a predictive value for the pathogenesis. It is important for dermatologists to recognize these cutaneous signs to permit early and accurate diagnosis and treatment.     

Cutaneous manifestations of Wegener's granulomatosis: a clinicopathologic study of 17 patients and correlation to antineutrophil cytoplasmic antibody status

BACKGROUND: Wegener's granulomatosis (WG), a systemic vasculitis, can be associated with cutaneous signs and symptoms before, during or after the diagnosis of systemic disease. METHODS: We reviewed clinical and histologic features of cutaneous lesions from 17 patients with WG. The temporal relationship between development of cutaneous symptoms and onset of systemic disease was determined, and antineutrophil cytoplasmic antibody (ANCA) status of the patients was also established. RESULTS: In six patients, systemic and cutaneous disease developed concurrently. In eight patients, cutaneous disease developed after patients received the diagnosis of systemic disease. In three patients, cutaneous disease preceded systemic disease. Cytoplasmic ANCA or proteinase-3-ANCA [c-ANCA/proteinase 3 (PR3)-ANCA] serologic test results were negative for one patient when cutaneous disease developed, and one patient had c-ANCA/PR3-ANCA seroconversion a year before systemic disease developed. Histopathologic features of cutaneous WG were not limited to leukocytoclastic vasculitis; they also included acneiform perifollicular and dermal granulomatous inflammation and palisaded neutrophilic and granulomatous inflammation. CONCLUSIONS: Patients with WG can present initially with cutaneous symptoms. Histopathologic patterns vary, but leukocytoclastic vasculitis is most commonly noted. Patients with WG and skin lesions are likely to have positive c-ANCA/PR3-ANCA serologic test results.     

Clinical Approach to Cutaneous Vasculitis

Vasculitis is an inflammatory process affecting the vessel wall and leading to its compromise or destruction and subsequent hemorrhagic and ischemic events. Vasculitis can be classified as a primary phenomenon (e.g. idiopathic cutaneous leukocytoclastic angiitis or Wegener granulomatosis) or as a secondary disorder (connective tissue disease [CTD], infection, or adverse drug eruption-associated vasculitis). Cutaneous vasculitis may present as a significant component of many systemic vasculitic syndromes such as rheumatoid vasculitis or anti-neutrophil cytoplasmic antibody (ANCA)-associated primary vasculitic syndromes (Wegener granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis). Cutaneous vasculitis manifests most frequently as palpable purpura or infiltrated erythema indicating dermal superficial, small-vessel vasculitis, and less commonly as nodular erythema, livedo racemosa, deep ulcers, or digital gangrene implicating deep dermal or subcutaneous, muscular-vessel vasculitis. A biopsy extending to the subcutis taken from the most tender, reddish or purpuric lesional skin is the key to obtaining a significant diagnostic result and serial sections are often required for identifying the main vasculitic lesion. Coexistence of pan-dermal small-vessel vasculitis and subcutaneous muscular-vessel vasculitis usually indicates CTD, ANCA-associated vasculitis, Behçet disease, or malignancy-associated vasculitis. A concomitant biopsy for direct immunofluoresence evaluation contributes to accurate diagnosis by distinguishing IgA-associated vasculitis (Henoch-Schönlein purpura) from IgG-/IgM-associated vasculitis, which has prognostic significance. Treatment for cutaneous vasculitis should include avoidance of triggers (excessive standing, infection, drugs) and exclusion of vasculitis-like syndromes (pseudovasculitis) such as thrombotic disorders (e.g. anti-phospholipid antibody syndrome). In most instances, cutaneous vasculitis represents a self-limited condition and will be relieved by leg elevation, avoidance of standing, and therapy with NSAIDs. For mild recurrent or persistent disease, colchicine and dapsone are first-choice agents. Severe cutaneous disease requires treatment with systemic corticosteroids or more potent immunosuppression (azathioprine, methotrexate, cyclophosphamide). A combination of corticosteroids and cyclophosphamide is required therapy for systemic vasculitis, which is associated with a high risk of permanent organ damage or death. In cases of refractory vasculitis, plasmapheresis and intravenous immunoglobulin are viable considerations. The new biologic therapies that act via cytokine blockade or lymphocyte depletion, such as the tumor necrosis factor-α inhibitor infliximab and the anti-B-cell antibody rituximab, respectively, are showing benefit in certain settings such as CTD and ANCA-associated vasculitis.     

Eruptions in life-threatening rheumatologic diseases

Dermatologic changes occur in a variety of rheumatic diseases. Skin can be the initial site of involvement, thus providing important clues for an accurate diagnosis based on cutaneous findings. Dermatologic findings can also be an indicator of systemic involvement and prognostic outcome; however, many connective tissue disorders have a wide variety of cutaneous manifestations, with significant overlap between different diseases. These skin signs often precede systemic clinical manifestations. Careful attention to characteristic dermatologic findings in Behçet’s disease, systemic lupus erythematosus, rheumatoid arthritis, and various vasculitis can provide prompt therapeutic approaches in the case of life-threatening complications of systemically involved rheumatologic diseases.     

Vasculitis

Many cutaneous and systemic disorders are associated with inflammation and necrosis of blood vessels. Several classifications of vasculitis have been used. Internists tend to utilize the classification of Fauci with modifications such as those by Cupps. Gibson and Ryan, who are dermatopathologists, have classified vasculitis based on vessel size, leukocyte type, and presence of granulomas. A more recent classification has been developed by Jennette, a pathologist, and colleagues. The etiology of vasculitis is varied; it includes bacteria, viruses, chemicals, autoimmune disease, malignancy and abnormal exogenous and endogenous proteins. Leukocytoclastic vasculitis can be experimentally reproduced by the Arthus phenomenon. IgM and C3 are found in cutaneous blood vessels and associated with circulating immune complexes. CH50, C3 and C4 may be reduced in serum. Increased incidence of nasal carriage of staphylococci is associated with higher relapse rates in Wegener's granulomatosis and toxic shock syndrome toxin from staphylococci is associated with the Kawasaki syndrome. Additionally, at least four systemic vasculitic drug reactions can be confirmed with patch testing. Antineutrophil cytoplasmic antibodies (ANCA) are found in association with certain systemic vasculitides. These may be tested with indirect immunofluorescence and enzyme linked immunosorbent assays (ELISA) with radioimmunoassays. Originally cytoplasmic ANCA (cANCA) was identified with proteinase 3 as the antigen and perinuclear ANCA (pANCA) was related to myeloperoxidase. While cANCA is very specific for proteinase 3, pANCA is associated with a number of antigens other than myeloperoxidase. pANCA is found with alcohol fixed but not formalin-fixed neutrophils. cANCA is particularly sensitive and specific for Wegener's granulomatosis and predicts prognosis and response to therapy. pANCA is not so specific and is associated with a number of other vasculitic syndromes. Cutaneous vasculitis is managed primarily with colchicine, dapsone and prednisone, with recent studies indicating that there may be a synergistic effect of pentoxifylline with dapsone. Systemic vasculitis involves treatment with various agents. Recently it has been observed that co-trimoxazole (trimethoprim/sulfamethoxazole) is useful in many cases of Wegener's granulomatosis along with other more toxic chemotherapeutic agents.     

Skin gangrene as an extraintestinal manifestation of inflammatory bowel disease

Inflammatory bowel diseases can commonly present many cutaneous lesions which can contribute to the diagnosis of the disease or its activity. The most frequent cutaneous or mucocutaneous manifestations suggesting ulcerative rectocolitis activity are erythema nodosum (3-10%), pyoderma gangrenosum (5-12%) and aphthous stomatitis (4%). Other reactive skin manifestations related to immunological mechanisms associated with the inflammatory bowel disease are: Sweet''s syndrome, arthritis-dermatitis syndrome associated with inflammatory bowel disease and leukocytoclastic vasculitis. We describe the case of a young man with diagnosis of ulcerative rectocolitis, which presented an extensive cutaneous gangrene secondary to microvascular thrombosis. The case represents a dermatologic rarity and should be recognized as a cutaneous manifestation related to the hypercoagulability state observed in the disease''s activity.     

Cutaneous pseudovasculitis

Cutaneous pseudovasculitis represents a heterogeneous collection of disorders that are capable of simulating cutaneous vasculitis and can be broadly classified into diseases that produce hemorrhage (petechiae, purpura, and ecchymoses) or vessel occlusion with resultant livedo, cyanosis, ulcers, digital necrosis, and/or gangrene. Overlap is not uncommon, but if present, one mechanism dominates. Hemorrhagic pseudovasculitis is due to vessel wall dysfunction (incompetence), which can be related to diverse factors that include vessel wall deposition of metabolic substances (amyloid, calcium), nutritional deficiencies (scurvy), nonvasculitic inflammatory purpura (pigmented purpuric dermatitis, arthropod, viral and drug reactions), degeneration of the vessel wall and supporting stroma (senile/solar purpura), direct vessel wall invasion of infective organisms, coagulation-fibrinolytic disorders (eg, thrombocytopenia), and vessel wall trauma. Cyanotic-infarctive pseudovasculitis is due vaso-occlusion by emboli, thrombi, or fibrointimal hyperplasia (endarteritis obliterans) and includes varied conditions such as purpura fulminans, Coumadin necrosis, antiphospholipid antibody syndrome, cardiac myxoma, cholesterol embolization, calciphylaxis, and radiation arteritis. Delayed and inappropriate diagnosis of pseudovasculitis leads to incorrect management and exposure to potentially deleterious treatment modalities such as corticosteroids and cytotoxic agents. The diagnosis of a pseudovasculitic disorder requires a high index of suspicion and should always be part of the differential diagnosis of vasculitis. Skin biopsy is a crucial step in differentiating pseudovasculitis from authentic vasculitis; absence of histologic evidence of vasculitis, particularly after multiple biopsies, should direct evaluation and diagnosis towards pseudovasculitis.     

Therapy of leukocytoclastic (necrotizing) cutaneous vasculitis

ABSTRACT: There are multiple therapeutic options for cutaneous necrotizing vasculitis. The choice of the proper therapy is made more difficult by a variety of competing classification systems, and by a paucity of adequate clinical trials to assess efficacy of the most commonly used therapies. This article outlines an approach to therapy based on the diagnosis, the type of cutaneous involvement, the presence or absence of extracutaneous findings, and the known risks and benefits of currently available therapies for vasculitis.