ﺑﺎﺯﮔﺸﺖ ﺑﻪ ﺻﻔﺤﻪ ﻗﺒﻠﯽ
خرید پکیج
تعداد آیتم قابل مشاهده باقیمانده : 3 مورد
نسخه الکترونیک
medimedia.ir

Evaluation of adults with cutaneous lesions of vasculitis

Evaluation of adults with cutaneous lesions of vasculitis
Literature review current through: Jan 2024.
This topic last updated: Jul 01, 2022.

INTRODUCTION — Cutaneous vasculitis comprises a diverse group of conditions characterized by acute, relapsing, or chronic inflammatory damage to small or medium-sized blood vessels in the skin or subcutaneous tissue. Cutaneous vasculitis can occur as a feature of multiple disorders and exhibits a wide variety of clinical manifestations. Examples of clinical findings include petechiae, palpable purpura, hemorrhagic bullae, nodules, ulcers, livedo reticularis, livedo racemosa, and urticaria.

Although cutaneous vasculitis can be a benign, transient condition, it may also be an indicator of underlying disease or systemic vasculitis. Careful assessment is essential for accurate diagnosis and optimal management. A typical initial evaluation includes a skin biopsy to confirm vasculitis, careful review of the patient history to assess for the etiology of vasculitis, and laboratory tests to assess for systemic involvement. When the cause of vasculitis is uncertain, additional tests may be helpful.

The general approach to the evaluation of adults with cutaneous lesions suggestive of vasculitis will be reviewed here. Overview discussions of vasculitis in adults and children and in-depth discussions of specific forms of vasculitis are provided separately. (See "Overview of and approach to the vasculitides in adults" and "Vasculitis in children: Evaluation overview" and 'Types of cutaneous vasculitis' below.)

TYPES OF CUTANEOUS VASCULITIS — The dermatologic addendum to the 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides provides a framework for the classification of vasculitides affecting the skin [1]. Major groupings are based upon the size of vessels involved, propensity to affect other organs, and associated causes:

Small vessel vasculitis (primarily affects dermal vessels [arterioles, capillaries, venules]):

Immune complex vasculitis:

-Immunoglobulin A (IgA) vasculitis (Henoch-Schönlein purpura) (see "IgA vasculitis (Henoch-Schönlein purpura): Clinical manifestations and diagnosis")

-Hypocomplementemic urticarial vasculitis (anti-C1q vasculitis) (see "Urticarial vasculitis")

-Cryoglobulinemic vasculitis (see "Overview of cryoglobulins and cryoglobulinemia")

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis:

-Microscopic polyangiitis (see "Granulomatosis with polyangiitis and microscopic polyangiitis: Clinical manifestations and diagnosis")

-Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) (see "Clinical features and diagnosis of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)")

-Granulomatosis with polyangiitis (see "Granulomatosis with polyangiitis and microscopic polyangiitis: Clinical manifestations and diagnosis")

Medium vessel vasculitis (primarily affects arteries in subcutaneous tissue):

Cutaneous polyarteritis nodosa (see "Cutaneous polyarteritis nodosa")

Polyarteritis nodosa (see "Clinical manifestations and diagnosis of polyarteritis nodosa in adults")

Variable-vessel vasculitis (may affect any type of vessel):

Behçet disease (see "Clinical manifestations and diagnosis of Behçet syndrome")

Cogan syndrome (see "Cogan syndrome")

Cutaneous single-organ vasculitis (skin-limited vasculitis that exhibits no association with systemic vasculitis):

Cutaneous immunoglobulin M (IgM)/immunoglobulin G (IgG) immune complex vasculitis (leukocytoclastic vasculitis with IgG/IgM deposits that does not fit into another defined immune complex vasculitis)

Nodular cutaneous vasculitis (erythema induratum of Bazin) (see "Erythema induratum (nodular vasculitis)")

Erythema elevatum diutinum (see "Erythema elevatum diutinum")

Additional defined variants include vasculitis associated with systemic disease (eg, rheumatoid vasculitis, lupus vasculitis, etc) and vasculitis associated with a probable etiology (ie, associated with a specific drug, infection, sepsis, neoplasm, etc).

Large vessel vasculitides, such as Takayasu arteritis and giant cell arteritis, typically do not affect vessels in the skin. Occasionally, giant cell arteritis results in cutaneous necrosis related to vascular compromise of vessels proximal to the skin [1]. Rarely, giant cell arteritis involves vessels in the panniculus [1]. Kawasaki disease, a form of medium vessel vasculitis, also does not typically involve vessels in the skin. (See "Overview of and approach to the vasculitides in adults", section on 'Major categories of vasculitis'.)

WHEN TO SUSPECT CUTANEOUS VASCULITIS — Suspicion for cutaneous vasculitis typically arises based upon the detection of suggestive cutaneous findings.

Suggestive findings — Cutaneous vasculitis may exhibit a variety of morphologies, which usually correlate with the pathologic processes occurring in the skin [2-4]. Clinical features can include manifestations typical of small vessel involvement (eg, petechiae, palpable purpura, hemorrhagic bullae, superficial ulceration, urticaria) or medium vessel involvement (eg, subcutaneous nodules, deep ulcers, livedo reticularis, livedo racemosa). The skin lesions are often asymptomatic but may be associated with pruritus, burning sensations, or pain.

Examination of the entire skin surface can be helpful for the assessment for cutaneous vasculitis. Cutaneous vasculitis most commonly occurs in a symmetrical distribution on the lower legs, dependent areas, or on areas of constrictive clothing due to increased hydrostatic pressure in these locations:

Petechiae – Petechiae are nonblanchable and nonpalpable, pinpoint macules (less than a few millimeters in diameter) that result from capillary inflammation and red blood cell extravasation (picture 1) [2]. Petechiae are nonblanchable due to the presence of extravasated erythrocytes in the dermis due to damaged vessel walls. Diascopy (the application of pressure to a skin lesion with a glass slide) is a helpful technique for identifying nonblanchable lesions.

Palpable purpura – Brisk inflammation of venules and arterioles initially manifests as infiltrated, erythematous papules and plaques. These progress to raised, nonblanchable (purpuric) lesions as damage to vessel walls increases (picture 2A-C) [2]. As with petechiae, diascopy can be used to confirm nonblanchable lesions.

Hemorrhagic bullae – Small vessel involvement in the dermis can result in necrosis of overlying skin with associated blisters and extravasation of red blood cells (picture 3) [2].

Subcutaneous nodules – Intense inflammation of medium-sized vessels (vessels with muscular walls in the deep dermis and subcutis) can lead to the formation of nodular lesions (picture 4).

Ulceration or digital necrosis – Ulceration and tissue necrosis occur when vasculitis results in reduced vascular perfusion in the skin (picture 5A-B) [2]. Superficial ulcers can occur in patients with small vessel vasculitis; deep ulcers are usually the result of medium vessel disease [5].

Livedo reticularis and livedo racemosa – Livedo reticularis presents as a localized or widespread, patchy, reticulated, vascular network with a red-blue or violaceous hue (picture 6A-B). It results from compromise of blood flow in medium-sized vessels and can occur in the setting of vasculopathy due to vasospasm, hypercoagulable states, thrombosis, increased blood viscosity, or embolic phenomena, as well as in association with vasculitis. Livedo racemosa presents with a more abrupt and broken vascular pattern than livedo reticularis (picture 7). It is strongly associated with Sneddon syndrome, a nonvasculitic disorder characterized by livedoid skin lesions and cerebrovascular accidents, but may also occur as a manifestation of medium vessel vasculitis and other disorders [6].

Urticaria – Unlike nonvasculitic urticaria, lesions of urticarial vasculitis usually persist for more than 24 hours and are frequently associated with a burning sensation or pain, rather than pruritus. Lesions may contain purpuric areas and can resolve with hyperpigmentation (picture 8). (See "Urticarial vasculitis".)

Mimickers — The presence of a petechial or purpuric eruption does not always indicate vasculitis. Examples of other disorders that may present with these findings include [7,8]:

Common vasculitis mimickers:

Pigmented purpuric dermatoses (eg, nonblanchable pinpoint macules, patches, or plaques, often on the lower legs) (picture 9) (see "Pigmented purpuric dermatoses (capillaritis)", section on 'Schamberg disease (progressive pigmentary purpura)')

Macular purpura due to chronic sun exposure, glucocorticoid therapy, trauma, or anticoagulants (picture 10)

Inflammatory disorders on the lower extremities or other dependent sites (eg, hemorrhagic macules or papules due to stasis dermatitis or maculopapular drug eruptions) (picture 11A-B)

Arthropod bites (eg, bedbugs) (picture 12)

Less common vasculitis mimickers:

Pernio (picture 13) (see "Pernio (chilblains)")

Scurvy (perifollicular hemorrhage) (picture 14) (see "Overview of water-soluble vitamins", section on 'Deficiency')

Platelet deficiencies or dysfunction (petechiae or macular purpura) (picture 15)

Hypercoagulable and thrombotic disorders (noninflammatory retiform purpura) (picture 16A-B) (see "Approach to the patient with retiform (angulated) purpura")

Cholesterol emboli (noninflammatory retiform purpura, digital gangrene, livedo reticularis) (picture 17) (see "Embolism from atherosclerotic plaque: Atheroembolism (cholesterol crystal embolism)")

Septic emboli (petechiae on the distal extremities, noninflammatory retiform purpura) (picture 18) (see "Complications and outcome of infective endocarditis", section on 'Metastatic infection')

Systemic amyloidosis (periorbital and pinch purpura) (picture 19) (see "Cutaneous manifestations of amyloidosis")

Strongyloidiasis (periumbilical purpura) (see "Strongyloidiasis")

Purpura fulminans (sharply demarcated retiform purpura in the setting of disseminated intravascular coagulation or sepsis) (picture 20) (see "Evaluation and management of disseminated intravascular coagulation (DIC) in adults" and "Clinical manifestations of meningococcal infection", section on 'Purpura fulminans')

Calciphylaxis (painful retiform purpura in adipose-rich areas, livedo reticularis) (see "Calciphylaxis (calcific uremic arteriolopathy)")

In addition, livedoid vasculopathy (also known as atrophie blanche) may be confused with vasculitis. Livedoid vasculopathy presents with punched-out ulcers on the lower legs with surrounding livedo reticularis or livedo racemosa (picture 21). (See "Livedoid vasculopathy".)

PATIENT ASSESSMENT — The evaluation of patients with cutaneous lesions of vasculitis focuses on confirming the presence of vasculitis, evaluating for extracutaneous involvement, and identifying the underlying cause. This typically involves consideration of a skin biopsy (or biopsies), careful review of the patient history and review of systems, and performance of select laboratory tests. A summary of the approach to patients with cutaneous features of small vessel vasculitis is provided in an algorithm (algorithm 1).

Skin biopsy to confirm vasculitis

Indications for biopsy — A diagnosis of cutaneous vasculitis may be strongly suspected based upon the physical examination; however, a biopsy is necessary for a definitive diagnosis.

In general, a skin biopsy for routine histopathologic examination should be performed whenever feasible. However, clinicians with expertise in the evaluation of cutaneous vasculitis may elect to delay a biopsy for patients with classic presentations of acute small vessel cutaneous vasculitis that have persisted for less than four weeks, have a clear removable or treatable inciting factor (eg, drug or infection), and exhibit no clinical or laboratory evidence of systemic involvement because many such cases resolve spontaneously within a few weeks. If the vasculitis fails to improve within four weeks (ie, new lesions developing or persistence of red or purple lesions), performance of a skin biopsy is indicated. Of note, residual, macular or patchy hyperpigmentation is common after resolution of vasculitis lesions.

When patients present with cutaneous features of small vessel vasculitis, an additional skin biopsy of lesional skin for direct immunofluorescence (DIF) may be performed at the same time as the biopsy for routine histopathologic examination to assess for IgA vasculitis. (See 'Direct immunofluorescence' below.)

Diagnostic criteria — A histologic diagnosis of cutaneous vasculitis is confirmed by the identification of findings that indicate an inflammatory process that results in damage to vessel walls [2,4]. Small vessels (venules and arterioles) require two out of three of the following criteria for a definitive diagnosis of vasculitis [9]:

Angiocentric and/or angioinvasive inflammatory infiltrates

Disruption and/or destruction of vessel walls by the inflammatory infiltrate

Fibrinoid necrosis (fibrin deposition within the vessel wall or lumen; results from the accumulation and conversion of plasma proteins [2])

Medium-sized vessels (small arteries and veins) in the deep dermis and subcutaneous tissue require both of the following criteria to confirm vasculitis:

Inflammatory infiltrate infiltrating the muscular vessel wall

Fibrinoid necrosis

Additional supportive findings — Other histopathologic findings that support but are not diagnostic for vasculitis include the presence of extravasated erythrocytes, nuclear debris (leukocytoclasia), necrosis of eccrine glands, endothelial cell damage swelling or necrosis, and cutaneous ulceration, infarction, or necrosis [9]. Findings that suggest particular forms of vasculitis (eg, granulomas in granulomatosis with polyangiitis or eosinophilic granulomatosis with polyangiitis [Churg-Strauss syndrome] and interface dermatitis in vasculitis associated with lupus erythematosus or dermatomyositis) represent additional helpful information that can be gleaned from a biopsy. "Leukocytoclastic vasculitis" is a pathologic term that describes the microscopic findings of a neutrophilic small vessel vasculitis, a characteristic feature of multiple forms of cutaneous small vessel vasculitis.

Procedure — Optimizing the timing of the biopsy, biopsy depth, and biopsy location increases the likelihood of identifying diagnostic features.

Timing — The histologic characteristics of vasculitic lesions evolve quickly. Selection of a lesion that the patient estimates has been present for 24 to 48 hours for biopsy may optimize both the detection of vasculitis and assessment of the type of inflammatory infiltrate, a feature that can be helpful for diagnosis. Lesions that are between 24 and 48 hours old are the most likely to demonstrate diagnostic findings. Biopsies of leukocytoclastic vasculitis taken prior to 24 hours are likely to have some infiltration of neutrophils but often do not yet exhibit fibrinoid necrosis. Beyond 48 hours, the inflammatory infiltrate in leukocytoclastic vasculitis begins to shift from a neutrophilic infiltrate to lymphocytes and macrophages, and then eventually clears, leaving only evidence of fibrinoid necrosis. (See 'Diagnostic criteria' above and 'Additional supportive findings' above.)

Type of biopsy — The cutaneous features dictate the type of biopsy that should be performed. In general, shave biopsies should be avoided, as they prevent the evaluation of vessels in the mid-dermis and deep dermis. (See "Skin biopsy techniques", section on 'Biopsy techniques'.)

The following principles are helpful for guiding the selection of the appropriate procedure:

Cutaneous findings suggestive of involvement of the vessels in the superficial to mid-dermis (petechiae, palpable purpura, or urticarial papules or plaques) should be evaluated with a punch biopsy that is 4 mm in diameter or larger. This allows for evaluation of vessels throughout the dermis.

Cutaneous findings suggestive of involvement of the vessels in the mid-dermis to the subcutaneous tissue (livedo racemosa, subcutaneous nodules, ulcers) require a large punch biopsy (8 to 10 mm) or wedge biopsy that includes the subcutaneous tissue.

Location — The cutaneous features determine the optimal location of the biopsy within the involved area. Biopsies of petechial lesions or palpable purpura can be taken from any site within the lesion.

The ideal location of the biopsy differs in patients with livedo racemosa or ulcerations. In livedo racemosa, biopsy should be performed within the pale center of an erythematous ring [2]. This is where the responsible vessel is likely to be located. When ulceration is present, the biopsy should be taken from the edge of the ulcer, rather than the ulcer itself. A biopsy taken from the base of an ulcer can reveal purely incidental findings of vascular injury that resemble the histopathologic findings of vasculitis [9]. Biopsies of nodular lesions should be centered over a tender nodule.

Biopsies of infarcted digits are generally low yield for evaluation for vasculitis, as the infarcted area is simply a manifestation of underlying vessel obstruction.

Biopsies of livedo reticularis, a feature that may accompany vasculitis, demonstrate nonspecific histopathologic findings and are usually not indicated.

Direct immunofluorescence — Although an additional skin biopsy for direct immunofluorescence (DIF) is commonly performed at the same time as the biopsy for routine histopathologic examination in patients with findings suggestive of small vessel vasculitis, an immediate biopsy for DIF is not mandatory when small vessel vasculitis is acute (duration <4 weeks) in the absence of symptoms or signs suggestive of systemic involvement because many of these cases resolve spontaneously. Reserving a biopsy for DIF for patients with small vessel vasculitis of unclear cause that persists for ≥4 weeks is a reasonable alternative. (See 'Biopsy for direct immunofluorescence' below.)

Evaluation for etiology and extracutaneous involvement

Initial assessment — The initial assessment focuses on identifying readily identifiable causes of vasculitis and systemic involvement. Appropriate initial steps include an assessment for risk factors for vasculitis and performance of a review of systems and select laboratory tests.

Review of risk factors — Although the cause of vasculitis can be elusive, the patient history is an important method for identifying the etiology of vasculitis. In particular, the history should assess for recent infections (particularly upper respiratory infections), drug exposure (including cocaine), connective tissue disease, and malignancy [2,9,10]. In a study that pooled data from over 2000 patients in studies that reported triggering factors or associated conditions in patients with vasculitis, the frequencies of idiopathic disease, infections, drugs, connective tissue disease, IgA vasculitis, malignancy, and primary systemic vasculitides were 39, 23, 20, 12, 10, 4, and 4 percent, respectively [9]. Other systemic disorders were less frequently reported. Rates of failure to identify the cause of vasculitis ranged from 3 to 72 percent of patients [9].

In particular, when evaluating for drug-induced vasculitis, the timing of the development of vasculitis can be helpful. Drug-induced vasculitis most often occurs 7 to 10 days after the introduction of the inciting medication, with the exception of drug-induced antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, which usually occurs a year or more after starting the causative medication [9].

Review of systems — Single-organ vasculitis limited to the skin is a diagnosis of exclusion. Patients should be questioned regarding signs or symptoms that could suggest systemic vasculitis or underlying disease (table 1). Examples of potentially helpful findings include:

Weight loss

Fatigue, chills, night sweats

Myalgias or arthralgias

Tea-colored urine or gross hematuria

Abdominal pain or melena

Chest pain

Dyspnea, cough, or hemoptysis

New-onset or worsening asthma

Nasal symptoms

Peripheral neuropathy

If these or other symptoms suggestive of systemic vasculitis or underlying disease are present, further testing guided by the specific symptoms present are indicated. (See "Overview of and approach to the vasculitides in adults", section on 'Clinical features suggestive of systemic vasculitis' and 'Additional studies' below.)

Laboratory tests — Routine initial laboratory tests suggested for all patients with cutaneous vasculitis include:

Complete blood count with differential and platelets – Cytopenias may suggest underlying connective tissue disease, leukocytosis can suggest infection or hematologic malignancy, and platelet abnormalities may reflect nonvasculitic causes of skin lesions.

Comprehensive metabolic panel – Evaluates for renal and hepatic abnormalities.

Urinalysis with microscopy – Identifies hematuria due to renal involvement.

Additional studies — Additional evaluation is indicated for patients in whom the cause of persistent vasculitis (duration ≥4 weeks) is unclear or who present with symptoms, signs, or laboratory results suggestive of extracutaneous involvement or underlying disease. This may include screening laboratory tests, other tests directed by patient symptoms or test results, and a skin biopsy for DIF (if biopsy for DIF was not already performed).

Laboratory and other tests — Typical laboratory tests obtained to evaluate patients with either persistent vasculitis of unknown cause or systemic symptoms include:

Hepatitis B and C serologies

Serum complement levels (total hemolytic complement [CH50], C3, and C4)

Antinuclear antibody (ANA) and anti-dsDNA, anti-Ro, anti-La, anti-RNP, and anti-Smith antibodies

Rheumatoid factor

Serum cryoglobulins

ANCA

HIV antibody

Positive ANCAs are helpful in the correct clinical context but do not confirm or eliminate a diagnosis of an ANCA-associated vasculitis. (See "Clinical spectrum of antineutrophil cytoplasmic autoantibodies", section on 'Disease associations'.)

Additional laboratory tests or imaging studies are selected based upon the results of these tests and the presence of worrisome signs or symptoms (table 1). For example, review of systems that detects weight loss, joint pain, sicca symptoms, oral or nasal ulcerations, hair loss, photosensitivity, or Raynaud phenomenon supports a thorough evaluation for underlying connective tissue disorders.

Pulmonary symptoms support performance of a chest radiograph and further evaluation as needed. ANCA-associated vasculitis can affect the small and medium vessels of the lung, causing inflammation and hemorrhage. Additionally, hypocomplementemic urticarial vasculitis syndrome, a subset of urticarial vasculitis, may present with hemoptysis, pleural effusion, and severe chronic obstructive pulmonary disease [11,12].

Unexplained weight loss, fevers, chills, and night sweats may signify a malignancy and should prompt further evaluation. However, an extensive search for malignancy is not warranted in patients without signs or symptoms suggestive of malignancy given the rarity of this association (less than 5 percent of all patients with cutaneous vasculitis [13-15]). Patients should be up-to-date on age-appropriate malignancy screening. Also, in addition to the standard complete blood count with differential, our evaluation of patients over the age of 60 who lack another identifiable cause of small vessel vasculitis includes serum protein electrophoresis and immunofixation and serum free light chains. Limiting imaging studies to those indicated based upon the presence of suggestive signs and symptoms is appropriate in this situation.

Biopsy for direct immunofluorescence — Direct immunofluorescence (DIF) is an essential component of the evaluation of cutaneous vasculitis in adults with small vessel vasculitis of unclear cause that persists for ≥4 weeks. DIF utilizes labeled antibodies to identify immunoglobulin and complement deposits within the skin.

Value — DIF can aid in the identification of the underlying etiology of the vasculitis. DIF is particularly important for the diagnosis of IgA vasculitis (Henoch-Schönlein purpura). Renal involvement is common in IgA vasculitis, warranting additional monitoring. Only the identification of perivascular IgA deposition with DIF confirms the diagnosis of IgA vasculitis [16]. (See "IgA vasculitis (Henoch-Schönlein purpura): Clinical manifestations and diagnosis", section on 'Diagnosis' and "IgA vasculitis (Henoch-Schönlein purpura): Management" and "IgA vasculitis (Henoch-Schönlein purpura): Kidney manifestations".)

DIF is also of value for distinguishing immune complex-mediated vasculitides (such as most cases of cutaneous leukocytoclastic vasculitis, IgA vasculitis [Henoch-Schönlein purpura], cryoglobulinemic vasculitis, urticarial vasculitis, and vasculitis secondary to autoimmune connective tissue disease) from pauci-immune vasculitis (granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis, and polyarteritis nodosa) (table 1) [17]. A negative DIF in the setting of histopathologically confirmed vasculitis should increase suspicion of these pauci-immune primary systemic vasculitides, and an appropriate work-up for systemic involvement should be undertaken. (See "Granulomatosis with polyangiitis and microscopic polyangiitis: Clinical manifestations and diagnosis", section on 'Diagnosis' and "Clinical features and diagnosis of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)", section on 'Evaluation' and "Clinical manifestations and diagnosis of polyarteritis nodosa in adults", section on 'Diagnosis'.)

Predominant deposition of IgM around vessel walls suggests the possibility of vasculitis related to type 2 or type 3 cryoglobulinemia or rheumatoid vasculitis (table 1). Patients with hypocomplementemic urticarial vasculitis may have deposition of IgG, complement, and fibrin on vessel walls, as well as along the dermal-epidermal junction [9]. (See "Clinical manifestations and diagnosis of rheumatoid vasculitis", section on 'Diagnosis' and "Overview of cryoglobulins and cryoglobulinemia", section on 'Biopsy' and "Urticarial vasculitis".)

Procedure — Ideally, DIF is performed on a lesion that is less than 24 hours old. The inflammatory reaction stimulated by the deposition of immunoglobulins in vessel walls quickly results in the destruction of both the vessel wall and the immune complexes, resulting in negative immunofluorescence.

In contrast to the biopsies for DIF performed for the evaluation of blistering skin diseases, which are taken from perilesional skin, specimens from vasculitic lesions should be taken from within the lesions. DIF studies cannot be performed on the formalin-fixed tissue obtained for hematoxylin and eosin staining, and a separate tissue specimen should be obtained.

Specimens can be placed in Michel's transport medium (a fixative) or normal saline, or can be flash-frozen with liquid nitrogen. Tissue submitted in saline must be processed within 24 hours. Examination of the specimen by a pathologist experienced in DIF microscopy is preferred.

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Vasculitis".)

SUMMARY AND RECOMMENDATIONS

Overview – Cutaneous vasculitis occurs as a result of inflammatory processes that target and damage small or medium-sized blood vessels in the skin (table 1). Cutaneous manifestations of vasculitis may include petechiae, palpable purpura, nodules, ulcers, digital necrosis, livedo reticularis, livedo racemosa, or urticaria. (See 'Types of cutaneous vasculitis' above and 'When to suspect cutaneous vasculitis' above.)

Etiology – A wide variety of disorders can lead to the development of cutaneous vasculitis. Infections and medications are the most common etiologies of cutaneous small vessel vasculitis. (See 'When to suspect cutaneous vasculitis' above and 'Evaluation for etiology and extracutaneous involvement' above.)

Patient assessment – The initial evaluation of patients with cutaneous lesions suggestive of vasculitis incorporates confirmation of the presence of vasculitis, assessment for the underlying cause, and the identification of extracutaneous involvement. This includes consideration of a skin biopsy, analysis of the patient history and review of systems, and basic laboratory tests (complete blood count with differential and platelets, comprehensive metabolic panel, and urinalysis with microscopy). A summary of the approach to patients with cutaneous features of small vessel vasculitis is provided in an algorithm (algorithm 1). (See 'Patient assessment' above.)

Role of skin biopsy:

Biopsy for routine histopathologic examination – A skin biopsy is required to confirm a diagnosis of cutaneous vasculitis. Ideally, biopsies for routine histopathologic examination should be taken from a lesion that is 24 to 48 hours old. The best type of biopsy and location for the biopsy depends upon the clinical manifestations. Histopathologic features of vasculitis include fibrinoid necrosis and an inflammatory infiltrate invading or damaging the vessel wall. (See 'Skin biopsy to confirm vasculitis' above.)

Biopsy for direct immunofluorescence microscopy – In patients with cutaneous features of small vessel vasculitis, an additional skin biopsy of lesional skin for direct immunofluorescence (DIF) may be performed at the same time as the biopsy for routine histopathologic examination to assess for IgA vasculitis (algorithm 1). Alternatively, if signs or symptoms of systemic involvement are absent, a biopsy for DIF may be reserved for patients in whom small vessel vasculitis persists for ≥4 weeks. (See 'Direct immunofluorescence' above.)

DIF testing can be a valuable tool for the diagnosis of small vessel vasculitis, as it can help to distinguish immune complex-mediated vasculitides from pauci-immune vasculitis. Ideally, the biopsied lesion should be less than 24 hours old. Biopsy specimens for DIF should not be placed in formalin. (See 'Biopsy for direct immunofluorescence' above.)

Patients with persistent vasculitis or signs of systemic involvement – Additional evaluation is indicated for patients with cutaneous vasculitis of uncertain etiology that persists for four or more weeks or who exhibit signs or symptoms suggestive of systemic vasculitis or an associated underlying disease (algorithm 1). For patients with vasculitis of uncertain etiology, this typically includes tests for hepatitis B and C, autoimmune disease, and other disorders associated with vasculitis and a skin biopsy for direct DIF testing (if not already performed). (See 'Additional studies' above.)

  1. 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. Arthritis Rheumatol 2018; 70:171.
  2. Carlson JA. The histological assessment of cutaneous vasculitis. Histopathology 2010; 56:3.
  3. Gonzalez-Gay MA, Garcia-Porrua C, Pujol RM. Clinical approach to cutaneous vasculitis. Curr Opin Rheumatol 2005; 17:56.
  4. Jennette JC, Falk RJ. The role of pathology in the diagnosis of systemic vasculitis. Clin Exp Rheumatol 2007; 25:S52.
  5. Xu LY, Esparza EM, Anadkat MJ, et al. Cutaneous manifestations of vasculitis. Semin Arthritis Rheum 2009; 38:348.
  6. Dhadly M, Dean SM, Eberhardt RT. Cutaneous changes in peripheral vascular arterial disease. In: Fitzpatrick's Dermatology in General Medicine, 7th ed, Wolff K, Goldsmith LA, Katz SI (Eds), McGraw-Hill, 2008. Vol 2, p.1667.
  7. Chung L, Kea B, Fiorentino DF. Cutaneous vasculitis. In: Dermatology, 2nd ed, Bolognia JL, Jorizzo, JL, Rapini RP, et al. (Eds), Elsevier Limited, 2008. Vol 1, p.347.
  8. Piette W. Purpura: Mechanisms and differential diagnosis. In: Dermatology, 2nd ed, Bolognia JL, Jorizzo JL, Rapini RP, et al (Eds), Elsevier Limited, 2008. Vol 1, p.321.
  9. Carlson JA, Ng BT, Chen KR. Cutaneous vasculitis update: diagnostic criteria, classification, epidemiology, etiology, pathogenesis, evaluation and prognosis. Am J Dermatopathol 2005; 27:504.
  10. Loricera J, Blanco R, Ortiz-Sanjuán F, 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 (Oxford) 2015; 54:77.
  11. Grotz W, Baba HA, Becker JU, Baumgärtel MW. Hypocomplementemic urticarial vasculitis syndrome: an interdisciplinary challenge. Dtsch Arztebl Int 2009; 106:756.
  12. Davis MD, Brewer JD. Urticarial vasculitis and hypocomplementemic urticarial vasculitis syndrome. Immunol Allergy Clin North Am 2004; 24:183.
  13. Solans-Laqué R, Bosch-Gil JA, Pérez-Bocanegra C, et al. Paraneoplastic vasculitis in patients with solid tumors: report of 15 cases. J Rheumatol 2008; 35:294.
  14. Fain O, Hamidou M, Cacoub P, et al. Vasculitides associated with malignancies: analysis of sixty patients. Arthritis Rheum 2007; 57:1473.
  15. Bachmeyer C, Wetterwald E, Aractingi S. Cutaneous vasculitis in the course of hematologic malignancies. Dermatology 2005; 210:8.
  16. Hung SP, Yang YH, Lin YT, et al. Clinical manifestations and outcomes of Henoch-Schönlein purpura: comparison between adults and children. Pediatr Neonatol 2009; 50:162.
  17. Minz RW, Chhabra S, Singh S, et al. Direct immunofluorescence of skin biopsy: perspective of an immunopathologist. Indian J Dermatol Venereol Leprol 2010; 76:150.
Topic 13769 Version 25.0

آیا می خواهید مدیلیب را به صفحه اصلی خود اضافه کنید؟