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 Table of Contents  
REVIEW ARTICLE
Year : 2022  |  Volume : 9  |  Issue : 1  |  Page : 1-9

Histopathology of vasculitis: Classification, controversies, and concepts


1 Department of Dermatology, Venkat Center for Skin and Plastic Surgery, Bangalore, Karnataka, India
2 Department of Dermatology, FRGUHS (Aesthetic Dermatology), Bangalore, Karnataka, India

Date of Submission26-Feb-2021
Date of Decision13-Nov-2021
Date of Acceptance21-Jan-2022
Date of Web Publication26-Oct-2022

Correspondence Address:
Anuradha Jindal
FRGUHS (Aesthetic Dermatology), 20-A, Model Town Extension, Ludhiana 141002, Punjab
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijdpdd.ijdpdd_22_21

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  Abstract 

Vasculitis comprises a diverse group of disorders with varying clinical, histopathological, and immunofluorescence findings arising due to an array of underlying pathogenic mechanisms. These varying reactions are primarily a reflection of complex interplay of different cells, vessels, and stroma. There are several controversies and differences of opinions in the diagnostic criteria, classifications, and terminologies. This article deals with different concepts and controversies in the subject, and also attempts to provide an algorithmic approach for the histopathological diagnosis.

Keywords: Lymphocytic, necrotizing, neutrophilic, nuclear dust, vasculitis


How to cite this article:
Mysore V, Jindal A. Histopathology of vasculitis: Classification, controversies, and concepts. Indian J Dermatopathol Diagn Dermatol 2022;9:1-9

How to cite this URL:
Mysore V, Jindal A. Histopathology of vasculitis: Classification, controversies, and concepts. Indian J Dermatopathol Diagn Dermatol [serial online] 2022 [cited 2023 Feb 8];9:1-9. Available from: https://www.ijdpdd.com/text.asp?2022/9/1/1/359689




  Introduction Top


Vasculitis involves the inflammatory processes that can affect small, medium-, or large-sized vessels. Several aspects of vasculitis including criteria for diagnosis, terminologies (eg. necrosis), and entities (such as lymphocytic vasculitis) have been the subjects of much debate and controversy leaving both a dermatologist and pathologist befuddled.

Vasculitis in the skin has traditionally been referred to as cutaneous small-vessel vasculitis, as this is the common type found and large vessel vasculitis, which usually does not usually involve skin.[1] It has also been known by synonyms such as cutaneous leukocytoclastic vasculitis, hypersensitivity angiitis/vasculitis variants confined to skin, cutaneous necrotizing venulitis (necrotizing variant involving predominantly venules).

Although the term vasculitis is used, routinely and commonly, the term is not defined precisely. Instead, criteria are listed as defining criteria for diagnosis.[2],[3],[4] In some textbooks, definition is mentioned, but often vaguely, as “inflammation of blood vessel walls.”[5],[6]

This article seeks to discuss the issues mentioned in Text Box 1 to achieve clarity and also outline an algorithmic approach for diagnosis. The objective of the article was not to discuss the specific features of each type of vasculitis, rather to outline concepts and approach. As an introduction, some commonly used terms are defined in Text Box 2 for the sake of clarity.

Text Box 1: Key points discussed in the article

Criteria for diagnosis of vasculitis

Necrotizing vasculitis- is it a correct term?

Vasculitis vs Perivasculitis

Lymphocytic vasculitis- is it an entity?


  Classification Top


There has been no consensus yet on a uniform classification.[7],[8],[9] Several classifications based on size and type of vessel and the type of cells have been proposed for vasculitis, but have not been found fully satisfactory.[7],[8],[9]

Text Box 2: Important definitions relevant for understanding vasculitis

Fibrinoid: PAS +ve material composed of fibrin, Ig and complement

Capillary: thin walled vessel with single layer of flat endothelial cell. They are distinguished from lymphatic vessels by the presence of RBCs in lumen

Venule: elongated larger vessel with single layer of endothelial cells, surrounded by connective tissue stroma (tunica externa).

Vein: endothelial cells lining the lumen, surrounded by smooth muscle cells, and connective tissue stroma.

Arteriole: thick walled round vessel, with three layers: tunica externa of connective tissue fibres, tunica media of muscle and tunica intima of endothelial cell.

Artery: In addition to the above layers, there is internal elastic lamina.

Lymph vessels: similar to capillary, without RBCs.

Nuclear Dust (leukocytoclasis): remnants of nuclei seen as bluish particles scattered in dermis. Seen in all types of leukocytoclastic vasculitis, Sweet’s syndrome.

Polymorphonuclear cells:

Appear as ‘salt and pepper’ in the dermis. The cell has a size of 10 microns with light pink cytoplasm, which contains granules and a multilobed nucleus.

The Chapel Hill consensus conference (CHCC) 2012 [Box 1] divides the blood vessels based on their structure and function rather than the diameter. Small vessels include intraparenchymal arteries, arterioles, capillaries, venules, and veins, whereas medium vessels comprise larger arteries and veins in the septae of the panniculus, main visceral arteries and veins, and their initial branches.[10]

Box 1: 2012 Revised International Chapel Hill Classification[

10]


Large vessel vasculitis

  Takayasu arteritis

  Giant cell arteritis

Medium vessel vasculitis

  Polyarteritis nodosa

  Kawasaki disease

Small vessel vasculitis

  Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis

  Microscopic polyangiitis

  Granulomatosis with polyangiitis (Wegener)

  Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

  Immune-complex vasculitis

  Anti-glomerular basement membrane disease

  Cryoglobulinemic vasculitis

  Immunoglobulin A (IgA) vasculitis (Henoch- Schönlein)

  Hypocomplementemic urticarial vasculitis (anti-C1q vasculitis)

Variable vessel vasculitis

  Behcet disease

  Cogan syndrome

Single-organ vasculitis

  Cutaneous leukocytoclastic angiitis

  Cutaneous arteritis

  Primary central nervous system vasculitis

  Isolated aortitis

  Others

Vasculitis associated with systemic disease

  Lupus vasculitis

  Rheumatoid vasculitis

  Sarcoid vasculitis

  Others

Vasculitis associated with probable etiology

  Hepatitis C virus-associated cryoglobulinemic vasculitis

  Hepatitis B virus-associated vasculitis

  Syphilis-associated aortitis

  Drug-associated immune complex vasculitis

  Drug-associated ANCA-associated vasculitis

  Cancer-associated vasculitis

  Others

Small vessel vs. large vessel vasculitis: A precise categorization and definition of vasculitis should take into account both vasculitis of small cutaneous blood vessels, that is, venules and arterioles (“small-vessel vasculitis”), and larger subcutaneous blood vessels, that is, veins and arteries (“large-vessel vasculitis”).[5] Capillaries are usually not involved primarily and specifically, in small vessel vasculitis and conditions involving capillaries have been separately referred to as capillaritis (e.g., Schamberg’s disease).

The diagnosis of “small vessel vasculitis” necessitates the presence of infiltration of inflammatory cells, along with fibrin in the wall of venules (and rarely, arterioles) and/or thrombi in the lumen of those vessels. On the contrary, in the case of large vessel vasculitis, the mere presence of inflammatory cells within the vessel wall is sufficient for a diagnosis. The reason is that diapedesis is common in small vessels because of their thinner walls, but is a less likely event in large caliber and thick-walled vessels and hence will not happen unless there is vasculitis. The inclusion of fibrin deposition and/or thrombi formation in case of small vessel vasculitis makes the diagnosis of vasculitis more specific excluding the other common routine causes of inflammation of small blood vessels.

However, there is a breach in this chronology. One exception to this criterion is “early small vessel vasculitis,” which exhibits only inflammatory cells in and around the blood vessel wall, whereas the process of fibrin deposition or thrombi formation is yet to unfold. This complicates the differentiation of early vasculitis from perivasculitis, an entity dealt with in more detail below.

Key messages: Changes in vasculitis are dynamic, temporal, and segmental, changing with time and with different parts of the lesion showing different changes.

For large vasculitis, the presence of inflammatory cells in the vessel wall is a sufficient criterion.

Criteria for small vessel vasculitis are more stringent, requiring the presence of inflammatory cells in vessel wall along with the evidence of damage to vessel wall (presence of fibrin and/or thrombi) [Box 2].

Neutrophilic vasculitis and nonneutrophilic vasculitis: Another approach to classify vasculitis is depending on the cell mediating the vasculitis: neutrophilic vasculitis and nonneutrophilic vasculitis.[11] Although neutrophilic vasculitis is easily definable by criteria (see below), nonneutrophilic vasculitis is a more heterogeneous group enclosing several entities such as lymphocytic vasculitis, granulomatous vasculitis, and eosinophilic vasculitis.

Box 2: Criteria for small vessel neutrophilic vasculitis

Criteria for small vessel neutrophillic vasculitis includes the following:[12]

  1. Damage to vessel wall leading to necrosis and hyalinization of vessel wall- hence called necrotizing vasculitis [Figure 1].


  2. Infiltration of vessel wall by inflammatory cells, usually neutrophils [Figure 2]


  3. Presence of nuclear dust (remains of Nuclei seen as dark blue particles) which indicate karyorrhexis and is called leukocytoclasis [Figure 3]


  4. Extravasation of RBCs [Figure 3]


  5. Deposition of Fibrinoid (PAS +ve material composed of fibrin, Immunogloulins and complement) in and around vessel [Figure 4]


  6. These changes are segmental
Figure 1: Leukocytoclastic vasculitis (H and E, ×10)

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Figure 2: Leukocytoclastic vasculitis with nuclear dust, necrosis of vessel wall (H and E, ×20)

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Figure 3: Leukocytoclastic vasculitis with nuclear dust, necrosis of vessel wall, and extravasation of RBCs (H and E, ×40)

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Figure 4: Perivasculitis- perimural infiltration of lymphocytes around vessels. (H and E, X10)

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Several aspects of these criteria need elaboration. These criteria are fully relevant to neutrophilic vasculitis but not so much to nonneutrophilic vasculitis. Therefore, much of the below discussion refers to neutrophilic vasculitis.

  1. Damage to vessel wall: It is a routine finding in a fully evolved lesion of small vessel vasculitis, whereas it is rarely seen in early lesions of the same. Evidence of destruction of vessel wall is also not routinely seen in large vessel vasculitis and lymphocytic vasculitis. Hence, although it is a useful criterion when present but its absence does not rule out vasculitis.


  2. Necrosis-necrotizing vasculitis: The word necrotizing vasculitis is frequently used synonymously for leukocytoclastic vasculitis (LCV). The term necrotizing angiitis, first coined by Zeek,[13] was proposed as a generic term for all types of vasculitis in which the fully developed stage is characterized by fibrinoid necrosis and inflammation involving all the three layers of vessel wall. The sequence of pathologic events in necrotizing venulitis of the superficial dermal plexus culminate in deposition of fibrillar and amorphous eosinophilic material within the vessel wall, which is infiltrated by polymorphonuclear leukocytes and finally becomes anuclear and necrotic.[14] Morphologically, the word necrotizing vasculitis is used to imply that the vessel wall undergoes damage, appears smudged and indistinct, and is filled with fibrin; it also leads to necrosis of the surrounding tissue due to ischemia.[15]


However, there are certain conceptual difficulties while using this term. The term necrosis is defined as irreversible cell death. Necrosis in vasculitis includes alterations in collagen fibers, as stated above, which is a nonviable fiber. Endothelial cell is the only resident cell of a venule, which swells up in the pathologic events of vasculitis but may not always undergo necrosis.

The only cells to undergo necrosis (karyorrhexis) in vasculitis are the neutrophils, as indicated by the term leukocytoclasis (nuclear “dust,” indicates necrosis of nuclei). However, neutrophils belong to lumen, not to the wall of the vessels. In fully developed lesions of leukocytoclastic vasculitis, deposition of fibrin material in the wall of the venules is often unjustifiably labelled as fibrinoid necrosis though no necrosis is happening. Definitely, occlusion of lumina in small-vessel vasculitis can lead to infarction, and infarction may produce necrosis of surrounding structures but this is a late event and also, might happen in other vasculopathies as well.

Text Box 3: Conditions included under leukocytoclastic vasculitis

  • Hypersensitivity vasculitis due to infections, drugs, connective tissue diseases, autoimmune disease


  • Henoch Schonlein purpura


  • Urticarial vasculitis


  • Cryoglobulinemic Vasculitis


  • Erythema elevatum diutinum


  • Granuloma fasciale


  • Rheumatoid neutrophilic vasculitis


  • Behcet’s disease


  • Septic vasculitis (e.g.: meningococcemia and gonococcemia)


  • Polyarteritis nodosa(PAN)


Thus, it is clear that the term necrotizing vasculitis is not exactly precise and is one of those terminologies in dermatology which continue to be used despite their vagueness and inaccuracy, much like the term necrobiosis and parapsoriasis.

Key message: The term necrotizing vasculitis is imprecise and should not preferably be used

Neutrophilic vasculitis in skin usually manifests clinically as a small-vessel vasculitis with predominance of neutrophils and involvement of venules predominantly. Neutrophilic vasculitis mainly includes immunologically mediated vasculitis (leukocytoclastic vasculitis) and nonimmunologically mediated vasculitis (eg. septic vasculitis).

  1. Leukocytoclastic vasculitis has a neutrophilic infiltrate with leukocytoclasis as the diagnostic feature [Figure 1][Figure 2][Figure 3] and is the prototype of cutaneous vasculitis. The conditions showing LCV are shown in Text Box 3.


  2. Septic vasculitis: The defining feature is presence of thrombi in the lumen of small vessels, without significant leukocytoclasis.


Arteriolitis

In addition to the above variants of cutaneous venulitis, arteriolitis can occur too, though less commonly, as in malignant atrophic papulosis (Degos’ disease). Plugging of the larger vessel arterioles leads to ischemia and a wedge-shaped degeneration of collagen throughout the dermis, which is soon replaced by wedge-shaped sclerosis. Mucin deposition accompanies these changes in the dermis. In the initial stages, neutrophils predominate which are replaced by lymphocytes gradually and the later stages are characterized by sclerosis without any inflammation.

Here it is important to clarify two terms that are frequently confused with vasculitis; vasculopathy and perivasculitits.


  Vasculopathy Top


Vasculopathy is used as an umbrella term to include all pathologies of vessels, although more practically it includes vascular disorders without any significant vessel wall inflammation, thus excluding vasculitis from its preview. Thrombus formation can occur without presence of above typical features of vasculitis. This group includes various entities such as Sneddon’s syndrome, consumptive vasculopathy associated with thrombus formation, hyperplastic disorders (e.g., pyogenic granuloma), benign and malignant neoplasias (e.g., angioma and angiosarcoma), hamartomas and malformations (e.g., nevus flammeus and aneurysms), and deposits and metabolic disorders (e.g., atherosclerosis).[2],[6 Livedoid vasculopathy],[ previously also termed as livedoid vasculitis is considered to be a vasculopathy as histologically it shows predominant hyalinization and thrombus formation without leukocytoclasis. Thus the term vasculopathy applies to a variety of disorders without specific connotation.

Key message: The term vasculopathy has been used in two contexts; to include all disorders of vessels or to include disorders of vessels excluding vasculitis


  Non-neutrophilic Vasculitis Top


As stated earlier],[ this is a heterogeneous group comprising of several entities.

  1. Lymphocytic vasculitis: Lymphocytic vasculitis has been a much-debated topic. Lymphocytes are found in several types of vasculitis],[ including the late lesions of classic LCV. Lymphocyte infiltration is seen in classic LCV lesions],[ after a few hours of the initiation of vasculitis and may be either it is an accompaniment or an epiphenomenon. The term lymphocytic vasculitis includes various conditions],[ of which pityriasis lichenoides et varioliformis acuta (PLEVA) is the prototype],[ where lymphocytes are the primary cells],[ seen in the earliest stage of the lesions. The conditions in this group also show some features of typical vasculitis],[ such as presence of cells within vessel wall],[ and edema which can distinguish from perivasculitis discussed above [Figure 4]. Even fibrin deposition may be seen],[ particularly in severe forms of PLEVA. Some authors have held the perspective that these criteria are adequate to define lymphocytic vasculitis as a specific entity.[11],[16] However, fibrinoid deposition and nuclear dust are not always a feature of lymphocytic vasculitis. Hence, other authorities have a contrasting view that the entity is difficult to define and lacks specific criteria.[17],[18],[19],[20],[21]


Some conditions showing lymphocytic vasculitis are shown in Text Box 4.

Text Box 4: Conditions included under lymphocytic vasculitis

  • Pityriasis lichenoides et varioliformis acuta


  • Progressive pigmented purpura


  • Rickettsial infections


  • Viral infections


  • Gyrate erythema.


  • Toxic erythema of pregnancy


  • Insect bites


  • Some drug eruptions


  • Kawasaki disease


Another classification[21] used vessels involved and accompanying morphological changes as diagnostic criteria for lymphocytic vasculitis: (1) lymphocytic endovasculitis, seen in perniosis, Kawasaki’s disease, and some cases of Dego’s disease; (2) lymphocytic lichenoid vasculitis, seen in PLEVA [Figure 5] and [Figure 6], lichenoid drug eruptions; and (3) angiocentric/angiodestructive lymphocytic vasculitis, seen in angiocentric lymphomas
Figure 5: Pityriasis lichenoides et varioliformis acuta showing focal parakeratosis, vaculation of keratinocyltes, and dense accumulation of lymphocytes around vessels in upper dermis (H and E, ×10)

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Figure 6: Pityriasis lichenoides et varioliformis acuta showing dense accumulation of lymphocytes around vessels in upper dermis; note the presence of lymphocytes in the vessel wall and extravasation of RBCs (H and E, ×40)

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Thus, although the entity lymphocytic vasculitis eludes consensus, it remains a useful terminology in routine practice to distinguish and identify specific entities such as PLEVA and hence the term continues to be used. It is also possible that better newer molecular biological techniques to identity the immunological sequence of events may offer a better tool for classification of this group.[21]

Key message: Lymphocytic vasculitis lacks adequate and several authors have questioned its existence. However, it is a useful concept in practice to distinguish some entities such as PLEVA, which show severe changes in vessel wall

  • 2. Granulomatous vasculitis: This is another term that needs proper elucidation. As by definition histiocyte is the primary cell of a granulomatous process, any diagnosis of granulomatous vasculitis, would need presence of histiocyte along with above-discussed criteria for vasculitis. The histiocyte is thought to play a paramount role in the causation and manifestation of Wegener’s granulomatosis,[22] allergic granulomatosis of Churg and Strauss, which are the prototypes of granulomatous vasculitis. However, majority of these conditions are systemic, and have minimal skin manifestations, except for rare cases of Wegener’s granulomatosis, and Crohn’s disease.[23],[24]


Granulomatous vasculitis seen in above conditions has to be differentiated from conditions in which co-existence of granuloma and vasculitis in the same lesion is seen, such as nodular vasculitis [Figure 7] and [Figure 8] (and its tubercular variant erythema induratum), necrobiosis lipoidica.[25],[26]
Figure 7: Nodular vasculitis showing presence of involvement of thick walled vessels in mid and lower dermis, occasional giant cell and panniculitis (H and E, ×20)

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Figure 8: Nodular vasculitis showing involvement of thick walled arteriole, filled with fibrin thrombus (H and E, ×20)

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Key message: Granulomatous vasculitis is applied to those conditions in which histiocyte has a primary role; however, in several conditions, both granuloma and vasculitis coexist

  • 3. Eosinophilic vasculitis: Eosinophils are frequently seen as a part of dermal infiltration in few sub-categories of vasculitis such as urticarial vasculitis and drug-induced vasculitis. Eosinophilic vasculitis, on the contrary, is a relatively newly described entity and exists either as an idiopathic condition or in association with other causes such as parasitic infection, hypereosinophilic syndrome, and connective tissue disease.[26],[27],[28] Both small and/or muscular vessels have been shown to be affected, resulting in digital necrosis in some cases. Direct immunofluorescence (DIF) studies in such cases have revealed angiocentric eosinophil major basic protein (MBP), peripheral neutrophil elastase staining, and absent mast cell tryptase staining, confirming the primary role for eosinophil.[29]


Key message: Eosinophilic vasculitis is a new entity in which eosinophils have a causative role

Key message: Neutrophilic reactions (Sweet’s syndrome) show some features of vasculitis such as dense neutrophilic infiltrate, nuclear dust, and dermal edema, but absence of vessel wall damage, accumulation of fibrin, and presence of thrombi excludes a diagnosis of vasculitis.


  Neutrophilic Vascular Reactions Top


Another issue causing confusion with vasculitis is the subject of neutrophilic reactions which are conditions characterized by predominantly neutrophilic dermal infiltrate, typified by Sweet’s syndrome. Some features of vasculitis such as accumulation of neutrophils, edema, and nuclear dust are present, but fibrin is not seen nor thrombi in lumina [Figure 9].[30] The infiltration is also more diffuse and often widespread throughout dermis. But as discussed earlier, initial stages of LCV might not show fibrin/thrombi formation and hence can lead to diagnostic confusion. One study proposed that presence of vasculitic changes need not exclude the diagnosis of Sweet syndrome and may be regarded as a variant of LCV.[31] Despite these findings, it can be guardedly said that presence of vessel wall damage, accumulation of fibrin, and presence of thrombi exclude a diagnosis of Sweet’s syndrome.
Figure 9: Sweet’s syndrome showing diffuse infiltrate of polymorphonculear cells, nuclear dust, but vessel wall damage and fibrin are absent (H and E, ×10)

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  Diagnostic Approach to Cutaneous Vasculitis Top


Having dealt with different terminologies, concepts, and controversies of vasculitis, we now delineate a simple histological approach to diagnosis of vasculitis. This includes

Step 1: Classify the type of vascular disorder: vasculitis, neutrophilic dermatoses, or obstructive vasculopathy.

Step 2: Identify the type of vessel involved (whether small, medium, or large vessel) [Algorithm 1].
Algorithm 1: Step 1 identification of type of blood vessel involved based on the clinical features

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Step 3: Identify the predominant inflammatory cell in the infiltrate, that is, neutrophils or nonneutrophils (lymphocyte, histiocyte, eosinophil) ([Algorithm 2] and [Algorithm 3])
Algorithm 2: Classification of vasculitis depending on the type of cell

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Algorithm 3: Classification of leukocytoclastic vasculitis depending on type of cell present

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Step 4: Identify special features which categorize the specific type of vasculitis [Table 1].
Table 1: Specific features of different types of vasculitis*

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An algorithmic approach is presented in the light of the above discussion [Algorithm 1][Algorithm 2][Algorithm 3][Algorithm 4]. Characteristic features of different entities showing cutaneous vasculitis are shown in [Table 1].
Algorithm 4: Medium/small-to-medium vessel vasculitis

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  Role of Biopsy Top


Biopsy should be performed from an early lesion to see typical changes. For immunofluorescence, biopsy should be preferably from a lesion within 12 h.[32] A biopsy taken after 48 h may show late changes, such as a lymphocytic infiltrate in a vasculitis which was previously a leukocytoclastic vasculitis.[33] One exception to this is chronic forms of LCV such as granuloma faciale (GF) and erythema elevatum diutinum (EED), which show LCV changes even in very late lesions.

The value of routine immunofluorescence is debatable.[34] It is useful only in some forms such as Henoch Schonlein vasculitis (HSV) (IgA deposition in the vessel wall) or Lupus erythematosus (deposition along basement membrane zone), cryoglobulinemia and hence is rarely necessary as a routine tool for diagnosis.[35],[36] Further results are dependent on age of lesions; late lesions often show negativity due to immunodegradation by inflammatory cells. A recent study by Sais et al.[37] showed that 84% of patients with cutaneous vasculitis had a positive direct immunofluorescence on biopsy. It has been suggested that a paucity of immune complexes suggests the presence of an Anti-neutrophilic cytoplasmic antibody-small vessel vasculitis (ANCA-SVV) and a negative DIF rules out ANCA.[38] The importance of ruling out ANCA-associated vasculitis is due to its multisystem involvement, especially renal, and watch out for associated diseases like SLE, Hepatitis B, Hepatitis C, and human immunodeficiency virus (HIV) or endocarditis.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
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    Figures

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Abstract
Introduction
Classification
Vasculopathy
Non-neutrophilic...
Neutrophilic Vas...
Diagnostic Appro...
Role of Biopsy
References
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