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Vasculitides



5  Non-tumorous skin diseases

5.10  Vasculitides

5.10.1  Leukocytoclastic vasculitis

Etiology, pathogenesis:

  • circulating immune complexes are deposited in the walls of small venules
  • complement activation attracts neutrophils
  • etiology in 50% unknown, infections, allergy, chronic autoimmune diseases

Clinical signs:

  • red palpable nodules
  • example: purpura Schönlein-Henoch
  • usually heals, severe vasculitis can cause ischemic damage of the epidermis

Pictures

Vasculitis allergica:
Vasculitis, CLINIC (1144)

Vasculitis, diabetes:
Vasculitis, CLINIC (1146)

Morbus Schönlein-Henoch:
Purpura Schönlein Henoch, CLINIC (901)

Vasculitis:
Idiopathic vasculitis, CLINIC (5980)

Further examples of allergic vasculitis:

Histology:

Neutrophils enter the walls of small venules, small fragments of nuclear debris are present (nuclear dust). Direct immunofluorescence: IgM, IgG, c3 within vascular walls, IgA perivascularly in purpura of Schönlein-Henoch.

Pictures

Leukocytoclastic vasculitis:
Leukocytoclastic vasculitis, HE 10x (438)

Leukocytoclastic vasculitis, HE 40x (439)

Another case:
Leukocytoclastic vasculitis, HE 20x (440)

Leukocytoclastic vasculitis, HE 40x (441)

Leukocytoclastic vasculitis, blue trichrome 40x (442)

Leukocytoclastic vasculitis, prominent exudation of fibrin:
Vasculitis leukocytoclastic, HE 40x (4305)
  [zoomify]

Leukocytoclastic vasculitis in a case of mixed cryoglobulinemia in a patient with C hepatitis:
Vasculitis leukocytoclastic, HE 40x (2511)

Vasculitis leukocytoclastic, HE 100x (2447)

Neutrophils are scarce sometimes:
Purpura of Schönlein-Henoch, HE 40x (379)

Purpura of Schönlein-Henoch, blue trichrome 40x (380)

Smudgy appearance of the tissue (caused by nuclear dust) is conspicuous in typical cases of leukocytoclastic vasculitis:
Purpura of Schönlein-Henoch, HE 20x (381)

Purpura of Schönlein-Henoch, HE 40x (382)

Another case, (blue trichome):
Leukocytoclastic vasculitis, blue trichrome 10x (297)

Another case:
Vasculitis leukocytoclastic, HE 10x (617)

Vasculitis leukocytoclastic, HE 40x (618)

Another case:
Vasculitis leukocytoclastic, HE 40x (3997)

Leukocytoclastic vasculitis:
Vasculitis leukocytoclastic, HE 20x (4636)
  [zoomify]

Leukocytoclastic vasculitis:
Vasculitis leukocytoclastic, HE 60x (13192)

Leukocytoclastic vasculitis:
Leukocytoclastic vasculitis, HE 60x (10681)

Leukocytoclastic vasculitis:
Leukocytoclastic vasculitis, HE 60x (10682)

Leukocytoclastic vasculitis:
Vasculitis leukocytoclastic, HE 60x (13270)

Leukocytoclastic vasculitis:
Vasculitis leukocytoclastic, HE 40x (12635)

Leukocytoclastic vasculitis:
Vasculitis leukocytoclastic, HE 40x (12636)

Vasculitis leukocytoclastic, HE 40x (12637)

Necrotizing vasculitis:
Vasculitis leukocytoclastic, HE 20x (4637)

Sometimes the inflammatory reaction is inconspicuous and hemorrhage dominates the picture (anticoagulation therapy, sometimes in combination with stasis, like this purpura located on the shins):
Purpura pigmentosa chronica, HE 20x (347)

Purpura pigmentosa chronica, HE 40x (348)

Case of slight vasculitic changes and prominent hemorrage in a patient with Warfarin anticoagulation therapy:
Vasculitis leukocytoclastic, HE 40x (4009)

Residual leukocytoclastic vasculitis:
Vasculitis leukocytoclastic, HE 40x (2373)

Immunofluorescence:
Vasculitis leukocytoclastic, anti-fibrin, FITC 40x (619)

Vasculitis leukocytoclastic, anti-fibrin, FITC 10x (620)

Vasculitis leukocytoclastic, anti-fibrin, FITC 40x (621)

Purpura Schönlein-Henoch:
Purpura of Schönlein Henoch, HE 100x (6111)

Purpura Schönlein-Henoch:
Purpura of Schönlein Henoch, HE 20x (5289)

Severe vasculitis, bullous:
Vasculitis bullous, HE 20x (5053)
  [zoomify]

5.10.2  Other vasculitides

Introduction:

Vasculitides are usually classified according to the size of the affected blood vessels and dominating inflammatory cells (neutrophilic leukocytoclastic, non-leukocytoclastic, lymphocytic, histiocytic). Small capillaries are affected in the group of diseases called progressive pigmented purpura or in stasis dermatitis, capillaritis can be found in a number of other diseases (e.g. gonococcal or meningococcal sepsis) and others. Diseases of large blood vessels (e.g. polyarteritis nodosa) are discussed elsewhere. Some of the vasculitic processes are granulomatous (e.g. Wegener's granulomatosis, arteritis temporalis of Horton).

5.10.2.1  Polyarteritis nodosa

Clinical signs:

  • palpable purpura
  • splinter hemorrhages
  • ulcerations
  • tender nodules
  • livedo reticularis
  • internal organs (kidneys) are affected, but some forms of PAN are limited to the skin

Histology:

Medium size and and small arteries show necrotizing vasculitis, thrombi, fibrin deposits, in later stages fibroblastic proliferation and granulation tissue, finally scarring, obliteration and recanalization. Sometimes aneurysmatic dilatation of the vessels may occurr.

Mixed infiltrate (neutrophils, lymphocytes, histiocytes, some plasmocytes, sometimes eosinophils); hemorrhage. Leukocytoclastic vasculitis of small cutaneous vessels is often present as well. Ischemia of supplied tissues may be present as well.

5.10.2.2  Eosinophilic vasculitis

Etiology:

  • primary, idiopathic (reason unknown)
  • secondary (Churg-Strauss syndrome, parazitic process etc.)
  • often within the scope of hypereosinophilic syndrome

Histology:

Vasculitis with many eosinophils in the infiltrate, often degranulated.

Pictures

Eosinophilic vasculitis (reason unknown):
Eosinophilic vasculitis, HE 60x (6387)

5.10.2.3  Churg Strauss syndrome

Clinical signs:

  • affects people with allergy
  • accompanied with eosinophilia
  • systemic vasculitis
  • affects lungs, subcutaneous tissue and other organs
  • subcutaneous nodules, purpura
  • rare

Histology:

Perivascular infiltrate with eosinophils, vasculitis with eosinophils, perivascular granulomas, thrombi. May heal with scarring.

5.10.2.4  Wegener's granulomatosis

Introduction:

Systemic disease affecting the blood vessels of the respiratory tract, kidneys, skin and sometimes of other organs as well. The process is characterized by necrotizing vasculitis with later formation of granulomas and ischemia of supplied tissues.

Clinical signs:

  • upper and lower respiratory pathways
  • glomelulonefritis
  • skin is affected at about half of patients
  • papulonecrotic lesions, especially above large joints
  • purpura, subcutaneos nodules, necrotizing granulomas and other
  • lethal without therapy

Etiology, pathogenesis:

Circulating ANCA antibodies (especially c-ANCA).

Histology:

Necrosis, perivascular fibrinoid degeneration, leukocytoclasia. Necrotizing vasculitis affecting small and medium dermal blood vessels. Bleeding. Later palisading granulomas with giant cells are formed.

Variable: neutrophils, sometimes eosinophils, thrombi, intimal proliferation in later stages.

Diagnosis is easier if the data of affected organs are available and laboratory examination confirms increased ANCA levels.

5.10.2.5  Thrombangiitis obliterans (Bürger)

Clinical signs:

  • rare, most often in young adults
  • male to female ratio 4:1
  • almost all patients are (heavy) smokers
  • afects any artery, but most common location are peripheral arteries on extremities (fingers, toes)
  • thrombosis, ischemia, necrosis, dry gangrene, amputation (or autoamputation)

Histology:

Thrombosis of small or medium arteries and (often as well) veins. Mixed perivascular infiltrate (lymphocytes, neutrophiles, eosinophiles, histiocytes and sometimes giant cells). Microabscesses. Later fibrosis and possibly recanalization.

Histology (especially in late stages) is not specific and clinical correlation is important.

5.10.2.6  Livedo vasculitis

Clinical signs:

  • ulcerations on lower extremities
  • age: adults
  • atrophia blanche develops later

Histology:

Thrombosed venules with fibrin in the dermis, slight lymphocytic inflammatory infiltrate, some neutrophils, bleeding, edema, epidermal necroses and ulcers.

5.10.2.7  Disseminated intravascular coagulopathy (DIC)

Etiology, pathogenesis:

  • generalized activation of blood coagulation
  • followed by fibrinolysis
  • both processes run simultaneously
  • platelet and plasma coagulation factors get consumed

Clinical signs:

  • tissue ischemia
  • bleeding

Histology:

Hyalin microthrombi in various stages of fibrinolysis. Usually some hemorrhage. Inflammatory changes are minimal. In some cases are epidermal necroses or subepidermal bullae.

Pictures

DIC, large bullae (pitva):
DIC, subepidermal bulla, HE 40x (6089)

5.10.2.8  Giant cell arteritis of Horton (temporal)

Clinical signs:

  • painfull arteritis
  • location: temporal
  • swelling, pain
  • may progress and affect nearby organs (eye)

Histology:

Arteritis with elastolytic granulomas and presence of giant cells; fibrosis, degeneration and mixed inflammatory infiltration with plasma cells, lymphocytes and histiocytes.

5.10.2.9  Purpura

Introduction:

Small capillaries are affected in a group of diseases calledProgressive pigmented purpura of Schamberg Majjocchi, in stasis dermatitis and other diseases (gonococcal or meningococcal sepsis).

Clinical signs:

  • area of predilection: lower extremities
  • skin hemorrhages: petechiae, maculae, papulae (palpable purpura), ecchymoses, suffusions
  • necroses, vasculitis
  • heals ad integrum or with residual pigmentations:

Pictures

Purpura:
Purpura, CLINIC (1125)

Purpura Gougerot-Blum, test:
Purpura Gougerot Blum, test with the turniket, CLINIC (899)

Morbus Schönlein-Henoch, purpura:
Purpura Schönlein Henoch, CLINIC (901)

Morbus Schamberg, purpura pigmentosa:
Purpura Schamberg, CLINIC (1074)

Vasculitis, purpura:
Vasculitis, CLINIC (1145)

Histology:

Purpura Majocchi-Schamberg is characterized by slight alteration of superficial capillaries with hemorrhage and perivascular lymphocytic infiltrate.

Purpura Schönlein-Henoch is classified as a vasculitis.

Pictures

Capillaritis of Schamberg purpura type:
Purpura pigmentosa chronica, HE 20x (346)

Another case:
Capillaritis, HE 20x (284)

Capillaritis, HE 40x (287)

Same case, special staining, green trichrome:
Capillaritis, Goldner 20x (283)

Capillaritis, Goldner 40x (286)

Purpura of Schamberg:
Purpura Schamberg, HE 20x (1671)

Purpura pigmentosa:
Progressive pigmented purpura, early stage, HE 40x (3583)

Another case, minimal inflammation:
Purpura pigmentosa chronica, HE 10x (344)

Purpura pigmentosa chronica, HE 20x (343)

Purpura pigmentosa chronica, HE 40x (345)

Purpura Schamberg:
Progressive pigmented purpura, early stage, HE 40x (4979)

Direct immunofluorescence (FITC), antibody against the fibrin (the same case as above):
Capillaritis, FITC 40x (285)

Capillaritis with admixture of neutrophils:
Capillaritis with neutrophils, HE 20x (288)

Capillaritis with neutrophils, HE 40x (289)

Lichen aureus, chronic purpura (HE and Perls for hemosiderin):
Lichen aureus, HE 40x (4373)

Lichen aureus, Prussian blue 40x (4372)

Lichen aureus, another case:
Lichen aureus, HE 40x (2112)

Lichen aureus, another case:
Lichen aureus, HE 20x (5113)

Lichen aureus, HE 40x (2361)

Lichen aureus, another case:
Lichen aureus, HE 60x (10684)

Another case of lichen aureus:
Lichen aureus, HE 40x (4134)

Lichen aureus, Prussian blue 40x (4133)

Another cases:
Lichen aureus, HE 20x (4332)

Lichen aureus, HE 20x (4333)

Lichen aureus:
Lichen aureus, HE 10x (1401)

Lichen aureus, HE 40x (1402)

Lichen aureus, HE 40x (1403)

Lichen aureus, HE and staining for iron:
Lichen aureus, HE 40x (6120)

Lichen aureus, Prussian blue 40x (6119)

Lichen aureus, HE and staining for iron:
Lichen aureus, HE 60x (6389)

Lichen aureus, Prussian blue 60x (6388)

Lichenoid purpura Doucas and Kapetanakis:
Lichenoid purpura, HE 40x (6033)

5.10.2.9.1  Thrombotic thrombocytopenic purpura of Moschowitz

Histology:

Superficial infiltrate (lymphocytes, histiocyte), superfical hemorrhage.

Pictures

Thrombotic thrombocytopenic purpura:
Thrombotic thrombocytopenic purpura of Moschkovicz, HE 20x (4304)

5.10.2.10  Urticarial vasculitis

Histology:

Papillary dermal edema, slight perivascular infiltrate with variable amount of neutrophils, sometimes slight leukocytoclasia, focal slight fibrin deposits of superficial venules, hemorrhage.

5.10.2.11  Vasculitis and mycotic sepsis

Clinical signs:

  • immunocompromized patients

Histology:

Variable perivascular infiltrate with neutrophils, necroses, fibrin deposits. Special staining shows intra- and perivascular mycotis spores and hyphae. Epidermal necroses are often present as well.

5.10.3  Chronic venous insufficiency

Clinical signs:

  • 1st grade: edema, corona phlebectatica
  • 2nd grade: hyperpigmentations, dermatoliposclerosis, atrophie blanche, stasis dermatitis
  • 3rd grade: venous ulceration or scar after ulcerations

Pictures

Chronic venous insufficiency, stem and reticular varices, prominent hyperpigmentations (hemosiderin deposits):
Stasis dermatitis: hyperpigmentation, varices, CLINIC (692)

Another examples of varicose veins and forms of chronic venous insufficiency:

Another examples of leg ulcerations:

Histology:

Superficial blood vessels have thickened walls, papillary corium contains groups of capillaries, sometimes spindle shaped endothelia are present as well; hemosiderin deposits and variable lymphocytic infiltrate. Superficial dermis is often fibrotic.

Epidermal changes are variable (atrophy, contact dermatitis etc.).

Sometimes irregular vascular proliferation can mimick Kaposi sarcoma (pseudo-Kaposi, acroangiodermatitis Mali).

Pictures

Stasis, acroangiodermatitis:
Stasis, acroangiodermatitis, HE 40x (6070)

Acroangiodermatitis Mali:
Acroangiodermatitis Mali, HE 20x (5077)
  [zoomify]

Acroangiodermatitis Mali, pseudokaposi:
Pseudo-Kaposi's sarcoma, HE 20x (5247)

5.10.3.1  Venous varices

Clinical signs:

  • stem varices: along large superficial veins (vv. saphenae), winded, dilated veins
  • reticular varices: smaller varices of the superficial plexus, corona phlebectatica
  • superficial varices
  • blow out: paplable dilatation in the site of insufficient oriffice of the perforator

Pictures

Stem varices: vena saphena magna:
Varix, CLINIC (939)

Stem varices:
Varix, CLINIC (940)

Varices:
Varices, CLINIC (5934)

Varices, CLINIC (5935)

5.10.3.2  Ulcus cruris

Clinical signs:

  • venous, postphlebitic ulcerations: above the inner ancle
  • arterial ulceration: foot, toes, tibial area, painful, necroses often
  • diabetic ulceration: fingers, soles, diabetic foot
  • ulcerations in hypertension: tibial area
  • another causes: vasculitic ulcerations
  • mixed ulcerations: combined causes

Pictures

Crural ulcerations:
Ulcus cruris, CLINIC (931)

Ulcus cruris venosum, atrophia alba, dermatoliposclerosis:
Ulcus cruris, CLINIC (932)

Ulcus cruris postphlebiticus:
Ulcus cruris, CLINIC (933)

Ulcus cruris, necrosis:
Ulcus cruris, CLINIC (934)

Ulcus cruris, granulations, epithelisation:
Ulcus cruris, CLINIC (1139)

Venous ulcus cruris:
Ulcus cruris, CLINIC (6369)

Histology:

Fibrin, nerosis, granulation tissue, sometimes with neutrophils, variable deposits of hemosiderin. The epidermis around is usually acanthotic (pseudoepithelimatous hyperplasia) and sometimes irritated (spongiosis, parakeratosis) as the reaction on extrenal therapy.

Pictures

Biopsy of the margin of chronic ulceration, prominent hemosidermis deposits, irritated epidermis:
Ulcus cruris, HE 40x (6041)

Ulcus cruris, Prussian blue 40x (6040)

Ulcus cruris:
Ulcus cruris, HE 60x (13793)

5.10.3.3  Superficial thrombosis

Clinical signs:

  • subcutaneous nodules
  • variable clinical settings with tendency to increased blood coagulation

Histology:

Dilated and thrombosed superficial veins.

Pictures

Superficial thrombosis:
Thrombosis, HE 20x (4609)

Venous thrombosis:
Venous thrombosis, HE 40x (5993)

Thrombosis, organized:
Thrombosis, organizing, HE 20x (5039)
  [zoomify]

5.10.3.4  Superficial thrombophlebitis

Clinical signs:

  • painful nodules
  • variable clinical settings with tendency to increased blood coagulation
  • varicous veins
  • migrating thrombophlebitis associted with cancers or Bürger's disease

Histology:

Dermal veins are filled by thrombi in variable stage of organisation. The wall of the vein as well as surrounding dermal connective tissue is inflammed. In acute cases there are usually many neutrophils.

5.10.4  Cutis marmorata, livedo reticularis, livedo racemosa

Clinical signs:

  • red and bluish net-like patches
  • initiated by cold conditions, usually subsides with warming
  • affects esp. children and women
  • location: most often legs

Etiology:

Variable, sometimes blood vessels disorders (vasculitis, antiphospholipid syndrome, drugs), sometimes no etiology can be identified. Immunofluorescence is sometimes slightly positive and slight perivascular lymphocytic infiltrates can be seen.

Histology:

Variable: dilatation of blood vessels, increased number of blood vessels, sometimes no changes can be found.

5.10.5  Cryoglobulinemia

Introduction:

Cryoglobulinemia is characterized by intravascular precipitation and obstruction.

Monoclonal cryoglobulinemia is associated with monoclonal proliferation, usually malignant: plasmocytoma (myeloma), immunocytoma and deposits consists of immunoglobulins (IgG, IgM). Inflammation is mild.

Mixed type sometimes accompanies chronic inflammatory diseases (rheumatoid arthritis, lupus erythematosus, hepatitis). Histology is dominated by leukocytoclastic vasculitis. Intravascular deposits are found only rarely.

Both types are characterized by ischemic lesions of acral parts of the body (ulcerations).

In some cases of cryoglobulinemia no primary process can be identified (essential cryoglobulinemia).

5.10.5.1  Cryoglobulinemia in monoclonal gamapathy

Introduction:

Affects patients with monoclonal gamapathy (Waldenström), most cases are patients with B lymphoma (immunocytoma).

Histology:

Homogenous eosinophilic material within dermal venules, dermal hemorrhare and often perivascular and sometimes interstitial infiltration with neutrophils.

5.10.5.2  Mixed cryoglobulinemia

Histology:

Usually characterized by leukocytoclastic vasculitis.

Pictures

Leukocytoclastic vasculitis in a case of mixed cryoglobulinemia in a patient with C hepatitis:
Vasculitis leukocytoclastic, HE 40x (2511)

Vasculitis leukocytoclastic, HE 100x (2447)

Cryoglobulinemia, mixed:
Cryoglobulinemia, type II., HE 20x (5090)
  [zoomify]

5.10.6  Acrocyanosis

Clinical signs:

  • hands and feet are affected
  • violet discoloration of fingers
  • attacs of pale discoloration of fingers
  • cold fingers

Pictures

Acrocyanosis in systemic scleroderma:
Acrocyanosis, CLINIC (652)

Acrocyanosis, small defects in systemic scleroderma:
Acrocyanosis, CLINIC (998)

Histology:

Histological picture is variable, forms with vasculitic changes are classified as vasculitides, in other cases the changes are secondary to scleroderma. Acrocyanosis of young persons (acrocyanosis crurum puellarum) has normal histological picture.

5.10.7  Malignant atrophic papulosis of Degos

Clinical signs:

  • affects usually younger individuals
  • more frequent in males
  • infarctions of the dermis, CNS, retina, GIT
  • skin lesions are usually asymptomatic, scarring, with teleangiectatic borders
  • usually progressive, lethal

Etiology:

  • unknown
  • cases affectins several members of a family were described

Histology:

Wedge shaped dermal infarctions, typically containing acid mucopolysaccharides, healing with sclerosis. Inflammation is minimal, thrombi within small vessels can be usually found.

5.10.8  Lymphedema

Clinical signs:

  • primary and secondary forms affect lower extremities, either symmetrically or unilaterally
  • subcutis is thickened, in long lasting lymphedema the change is irreversible
  • the skin does not wrinkle, dimpling is not possible
  • erysipelas is frequent complication of lymphedema

Pictures

Verrucosities in advanced secondary lymphedema:
Lymphedem, CLINIC (801)

Microbial eczema and lymphedema:
Eczem dermatitis, CLINIC (1020)

Primary lymphedema of the lower extremity:
Lymphedem, CLINIC (1073)

Histology:

Minimal finding, sometimes slight dilatation of lymphatic vessels.

Pictures

Lymphedema (according to clinical correlation):
Lymphedem, HE 40x (4728)

Lymphedema:
Lymphedem, HE 40x (13197)

5.10.9  Lymphangitis

5.10.9.1  Acute lymphangitis

Clinical signs:

  • associated with some focus of purulent inflammation
  • inflammation spreads through lymphatic vessels; often accompanied by phlegmone
  • red stripes on the skin
  • painful, enlarged lymph nodes

Histology:

Dilated lymphatic vessels with neutrophils; phlegmone.

5.10.9.2  Sclerosing lymphangitis of the penis

Clinical signs:

  • Nearly asymptomatic, firm, cordlike lesion of the penis in the coronal sulcus

Pictures

Sclerosing lymphangitis of the penis:
Lymphangitis sclerotisans, CLINIC (3113)

Histology:

Dilated lymphatic vessel containing fibrin thrombus, later replaced by connective tissue (histiocytes, fibroblasts, lymphocytes).



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