Cutaneous Vasculitis

1. Dr.Yugandar

2.  Is an inflammation & necrosis of blood vessels
resulting in a wide variety of clinical manifestations
 Cutaneous & systemic
 Small vessel vasculitis is characterized by palpable
purpura on dependent parts ie ankle & lower legs
 Large vessel vasculitis is characterized by necrotizing
livedo reticularis or multiple sites of peripheral
gangrene.

3.  Inflammatory destruction of the wall of blood vessel
by leukocytes  obstruction of the lumen  ischemia
& infarction of the tissues supplied.
 Triggered by various factors: infection, medications,
underlying diseases such as connective tissue diseases,
IBD, myelodysplastic & malignancies.

4. CLASSIFICATION
ZECK differentiated necrotizing angitis:
1) Periarteritis nodosa
2) Hypersensitivity arteritis
3) Rheumatic arteritis
4) Allergic granulomatous angitis
5) Temporal arteritis

5.  American college of rheumatology criteria for
hypersensitivity vasculitis:
 3 of 5 criteria has 83 % specificity & 71 % sensitivity.
1) Age at disease onset > 16 yrs
2) Medication at disease onset
3) Palpable purpura
4) Maculopapular rash
5) Biopsy including arteriole & venule with histological
changes showing granulocytes in a perivascular or
extra vascular location

6.  1994, CHAPEL HILL CONSENSUS CONFERENCE,
1) LARGE VESSEL VASCULITIS:
a) Giant cell arteritis
b) Takayasu’s arteritis
2) MEDIUM VESSEL WALL VASCULITIS:
a) classic polyarteritis nodosa
b) kawasaki disease
c) nodular vasculitis
3) SMALL VESSEL VASCULITIS:
a) Wegener's granulomatosis
b) churg-strauss syndrome
c) microscopic polyangitis

7. d) Henoch-schonlein purpura
e) Essential cryoglobulinaemia
f) Cutaneous leukocytoclastic vasculitis
g) Drug induced vasculitis
h) Other syndromes:
i) Acute hemorrhagic edema of infancy
ii) Urticarial vasculitis
iii) Serum sickness
iv) Erythema elevatum diutinum
v) Granuloma faciale
vi) Eosinophilic vasculitis

8.  1976, upgraded version of GILLIUM’S, proposed
working classification of vasculitis:
a) SMALL VESSEL VASCULITIS
1) Cutaneous small vessel vasculitis
2) H-S purpura
3) Essential mixed cryoglobulinaemia
4) Urticarial vasculitis
5) Erythema elevatum diutinum
6) waldenstorm’s hypergammaglobulinaemic purpura

9. 7) Eosinophilic vasculitis
8) Rheumatoid nodules
9) Reactive leprosy
10) Septic vasculitis
b) LARGE VESSEL VASCULITIS:
1) Polyarteritis nodosa
 Micoscopic polyarteritis
 Cutaneous form
 Systemic form

10. 2) Granulomatous vasculitis:
 Wegener’s granulomatosis
 Churg-strauss syndrome
 Lymphomatoid granulomatosis
3) Giant cell arteritis
 Temporal arteritis
 Takayasu’s arteritis
4) Large vessel vasculitis with collagen vascular diseases
5) Nodular vasculitis

11. PATHOGENESIS
 The proposed mechanisms for production of vascular
inflammation include:
1) Hypersensitivity reactions mediated by immune
complexes
2) Auto antibodies
3) Cellular immune responses

12. ROLE OF PATHOGENIC IMMUNE COMPLEXES
ANTIGENS {MICROBES, DRUGS, TUMOUR, AUTOANTIGENS}
ANTIBODIES {FORMATION OF IMMUNE COMPLEXES
DEPOSITION OF COMPLEXES IN & AROUND THE BLOOD VESSELS
ACTIVATION OF COMPLEMENT CASCADE & GENERATION OF C3 A& C5 A
{ ANAPHYLATOXINS}

13. MAST CELL DEGRANULATION & GENERATION OF
CHEMOKINES & CYTOKINES
INCREASED VASCULAR PERMEABILITY, NEUTROPHIL CHEMOTAXIS,
FURTHER DEPOSITION OF IMMUNE COMPLEXES
APPEARANCE OF NEUTROPHILS WITH PHAGOCYTIC ACTIVITY &
RELEASE OF PROTEOLYTIC ENZYMES {COLLAGENASE & ELASTASE}
DESTRUCTION OF VESSELS , FORMATION OF PLATELET THROMBI
ICHAEMIA, HAEMORRHAGE & NECROSIS OF TISSUES INVOLVED
CLINICAL SIGNS & SYMPTOMS
BY ENDOTHELIAL CELLS
IL-1,6,8
TNF-α
GM-CSF

14. ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES
{ANCA}
 Are antibodies directed against specific cytoplasmic
proteins of neutrophils & are present mainly in the
sera of patients with systemic vasculites & certain
other systemic disorders like connective tissue
diseases, IBD & auto immune liver diseases.
 Seen in wegener’s granulomatosis, microscopic
polyangitis & churg-strauss syndrome, drugs like
propyl thiouracil.

15.  Detected by the IIF test.
 Alcohol fixed indicator neutrophils are treated with
the patients sera & examined for patterns of IF.
 2 main types:
1) Diffuse staining of the cytoplasm- C ANCA
2) Staning Around The Nucleus- Perinuclear ANCA, P
ANCA

16.  The specific antigenic protein recognized by C ANCA
is, proteinase 3, a serine proteinase present in the
primary granules of neutrophils, designated as PR3
ANCA.
 P ANCA s are directed against myeloperoxidase as well
as other proteins present in the cytoplasm of
neutrophils, like elastase, cathepsin & lactoferrin,
MPO ANCA

17. CELL MEMBRANE EXPRESSION OF THE TARGET ANTIGENS, MAKING
THEM ACCESSIBLE FOR ANCA
PRIMING & APOPTOSIS OF NEUTROPHILS
DEGRANULATION & OXIDASE BURST OF NEUTROPHILS
ACTIVATION OF MONOCYTES , INDUCTION OF EXPRESSION OF
ADHESION MOLECULES ENHANCEMENT OF ADHESION NEUTROPHILS
& MONOCYTES TO ENDOTHELIAL CELLS
DESTRUCTION OF VESSELS

18. ANTIENDOTHELIAL CELL ANTIBODIES {AECA}
 Role in the pathogenesis of takayasu’s arteritis &
kawasaki disease.
ENDOTHELIAL DAMAGE BY ANTIBODY MEDIATED CELLULAR
CYTOTOXICITY WITH OR WITH OUT PARTICIPATION OF COMPLEMENT
INCREASED EXPRESSION OF ADHESION
MOLECULES
DESTRUCTION OF VESSELS
MIGRATION OF INFLAMMATORY CELLS

19. Cellular immune response & delayed
hypersensitivity
 Important in granuloma formation in large vessel
vasculitis like takayasu’s arteritis & giant cell arteritis
 Predominant accumulation of interferon gamma
producing CD4 + T lymphocytes (Th1 type cells) 
granuloma formation with giant cells.

20. CUTANEOUS LEUKOCYTOCLASTIC VASCULITIS
 Cutaneous small vessel vasculitis, hypersensitivity angitis &
necrotizing vasculitis.
 Most common vasculitis, affects both children & adults.
 Common in women.
 Affects cutaneous post capillary venules.
 It includes palpable purpura, urticaria , ulcers on leg.
 Palpable purpura starts as macular erythema & progress to
become papules, nodules, vesicles , bullae, pustules,
annular lesions or necrotic ulcers.

22.  Heals with post-inflammatory hyper pigmentation.
Seen in dependent areas , mainly the lower legs, ankles
associated with burning sensation , pruritis & pain.
 Systemic features : arthralgia, glomerulo-nephritis &
abdominal complaints.
 H/O drug intake or an infection prior to onset of rash.
 Predominantly limited to the skin in their clinical
expression , thus excluding those cases of cutaneous
vasculitis affecting other organ systems.

23. Secondary forms of cutaneous vasculitis
DRUGS : sulfonamide, minocycline, penicillin, thiazides,
NSAIDS, barbiturates, gold salts, Allopurinol,
propylthiouracil, methimazole, indinavir.
 Drug induced vasculitis develops with in 7-21 days after
treatment begins.
 Signs & symptoms are generally limited to skin.
 Systemic symptoms – fever, malaise, polyarthralgia.
 Cutaneous :- palpable purpura, ulcer, urticaria &
hemorrhagic blister.

26. INFECTIONS : Streptococcal, staphylococcal infections,
syphilis, TB, leprosy, gonorrhea, Hepatitis – A,B & C,
HSV, CMV, EBV, HIV, Candidiasis.
 Microbial antigens induces immune complex
formation leads to damage to vessels.
Viral infections of endothelia
Cross reactivity between endogenous & viral antigens
Immune reactions, viral tropism to b lymphocytes

27. CIRCULATING IMMUNE COMPLEXES CONTAINING
CRYOGLOBULINES
DAMAGE TO BLOOD VESSELS
AUTOANTIBODY PRODUCTION
B CELL PROLIFERATION

28. MALIGNANCIES :
 Hematological: leukaemia, hodgkin’s & non hodgkin’s ,
multiple myeloma
 Solid organ malignancies: carcinoma of prostate, breast,
colon, ovary, squamous cell carcinoma of lung & renal
cancer.
AUTOIMMUNE DISEASES:
 SLE, rheumatoid arthritis, sjogren’s syndrome,
dermatomyositis
OTHER SYSTEMIC DISEASES:
 α 1 anti trypsin deficiency, IBD, behcet’s diseases, chronic
active hepatitis, sarcoidosis

29. HENOCH-SCHONLEIN PURPURA
Syn: Ig A immune complex vasculitis, anaphylactoid
purpura, purpura rheumatoide.
 Vasculitis affecting small vessels, that
characteristically involves the skin, gastrointestinal
system & glomeruli with or without arthralgia or
arthritis.

30.  In response to
- infections with streptococci, mycoplasma, EBV,
varicella
- insect bites, vaccinations like typhoid, cholera,
measles & yellow fever
- drugs: pencillins, macrolides & aspirin

31. CRITERIA:
 Palpable purpura
 Bowel angina
 GI bleeding
 Hematuria
 Age of onset < 20 yrs
 No medication as a precipitating agent
3 or more

32. Clinical features
 Commonest childhood vasculitis
 Cutaneous:
erythematous urticarial papules which may evolve
with in 24 hrs into palpable purpura with hemorrhage,
over the extensor aspects of the limbs & buttocks in
symmetrical fashion.
 Crops of new lesions can recur for a few wks to several
months & fade with in 5-7 days.

34.  Renal: proteinuria, hematuria & red blood cell casts
 GIT: abdominal pain, nausea, vomitings, diarrhea &
passage blood in stools.
 Scrotal involvement with severe pain present.
 Most of the patients have polyarthralgia.

35. URTICARIAL VASCULITIS
 Hypo complementaemic urticaria- vasculitis
syndrome, unusal SLE like syndrome
 Chronic disease, which presents as urticarial lesions
over the trunk, proximal limbs, associated with
angioedema.
 2 types:
1) UV with Hypo complementaemia
2) UV without Hypo complementaemia

36.  Hypo complementaemic vasculitis: defined by the
presence of anti-c1 q precipitin & or decrease in the
level of c1.
 Seen in females
A/W :
 SLE, sjogren’s syndrome, physical urticaria, HBV, HCV,
serum sickness, colon cancer & drug ingestion.
 Exercise & with exposure to UV light & cold.
 Type III hypersensitivity

37. Clinical features
 Erythematous indurated wheals, contains purpuric foci.
 Angioedema & macular erythema seen.
 Livedo-reticularis, nodules & bullae also seen .
 Constitutional symptoms: fever, malaise, myalgia,
lymphadenopathy, hepatosplenomegaly, abdominal
pain with / with out nausea, diarrhoea, laryngeal
edema, dyspnoea, COPD, glomerulonephritis &
conjunctivitis.

39. CRYOGLOBULINEMIC VASCULITIS
 Vasculitis mediated by immune complexes containing
cryoglobulins.
 Cryoglobulins are circulating immunoglobulins or
complexes containing immunoglobulins that
precipitates in the cold & dissolves on rewarming.
 Cryoglobulins are classified according to their
constituents:
Type I cryoglobulins: monoclonal Ig associates with
multiple myeloma & other para proteinemias

40. Type II cryoglobulins: is a complex of monoclonal
component with polyclonal Igs.
Type III cryoglobulins: heterogenous mixture of
polyclonal Igs & non immunoglobulin molecules like
complements or lipoproteins.
 Type ii & Type III -mixed cryoglobulins.
 Associates with HCV, HBV, HIV, SLE, RA, lymphomas
& leukamias

41. Clinical features
 Common in women.
 Age of onset-5th decade
 Cutaneous: palpable purpura limited to lower limbs.
 Livedoreticularis, urticarial lesions, ecchymosis, acral
necrosis & leg ulcers are common.
 Systemic: weakness, arthralgia, polyneuropathy, raynaud’s
phenomenon, sicca syndrome & renal involvement

42. ERYTHEMA ELEVATUM DIUTINUM
 Rare chronic cutaneous eruption seen in adults.
 Characterized by fibrosing plaques with histological
evidence of leukocytoclastic vasculitis
 A/W : HIV, celiac disease, streptococcal infection &
monoclonal gamopathies.

43.  Clinical features: papules plaques & nodules, initially
soft, reddish & later becomes fibrosed & forms
atrophic scars in symmetrical fashion over the dorsa of
the hands, knees, buttocks & achillis tendon.
 Systemic features present
 D/D : sweet’s syndrome, in character of lesions & their
distributon.

44. EOSINOPHILIC VASCULITIS
 Is a cutaneous vasculitis of uncertain etiology
characterized by prominent necroizing vasculitis with
eosiophils on histology.
 Purpuric papules & urticarial lesions over the face &
extrmities.
 Systemic involvement is absent.

45. GRANULOMA FACIALE
 Common in males
 A rare dermatitis of uncertain etiology clinically
presenting as asymptamatic nodules & plaques
involving the face without any systemic involvement
 histologically characterized by a polymorphous
infiltrate with evidence of leukocytoclastic vasculitis.
 Lesions are smooth surfaced with prominent follicular
orifices & telangiectasia on the surface.

46. ACUTE HAEMORHAGIC EDEMA OF INFANCY
 Finkelstein’s disease.
 Characterized by an acute illness consisting of a
symptom triad of fever, large purpuric skin lesions &
peripheral edema.
 Children < 2yrs of age, with upper respiratory tract
infection.
 Precipitating: CMV, HAV, & drugs.

47.  Disease starts acutely with large , annular & targetoid
purpuric lesions involving the extremities & face.
 An acral edema develops & spreads proximally.
 Fever accompanies the rash & edema.
 D/D : HS purpura

48. SERUM SICKNESS
 Acute hypersensitivity reaction defined as occuring in
association with administration of a heterologous or
foreign proteins or serum.
 Serum sickness like reactions seen with cefaclor,
penicillin, streptokinase, minocycline &
cotrimoxazole.
 Others: blood products, hormones, vaccinations &
insect venoms.

49. Clinical features:
 begins 6-21 days after administration.
 Consists of skin rash together with fever, arthralgia &
lymphadenopathy
 Cutaneous: purpuric, EMF like, maculo-papular eruptions.
 Systemic associations are renal, pulmonary & neurological
manifestations.
 Self limiting
 D/D: EMF, SLE, urticarial vasculitis, viral exanthem

50. WEGENR’S GRANULOMATOSIS
 Triad consisting of systemic small vessel vasculitis,
necrotizing granulomatous inflammation of both the
upper , lower respiratory tracts & glomerulonephritis.
 If not treated, leads to end organ damage & then death
 Chronic nasal carriage of staphylococcus aureus
 Mean age of onset -40 yrs & both sexes

51. Cutaneous:
 Palpable purpura
 Lesions may be papular, plaque like, ulcerated,
vesicular, urticarial, subcutaneous nodules &
panniculitis
 Strawberry gingival hyperplasia – due to
granulomatous surface
 Constitutional: weakness, weight loss, anorexia &
arthralgia.

52.  URTI: Sinus Pain, Purulent sinus discharge, bloody
sinus discharge, nasal ulceration with epistaxis &
otitis media.
 Serious manifestations: tracheal inflammation,
sclerosis of the subglottic region  stridor & airway
obstruction, nasal septal perforation with saddle nose
deformities & facial paralysis
 Lung: cough, dyspnoea, hemoptysis & chest pain

53.  Glomerulonephritis & renal disease – mortality
 Eye : conjunctivitis, sclertis, uveitis
 Cardiac: cardiomyopahy, pericarditis &CVD
 CNS: mononeuritis multiplex, cranial neuritis &
cerebral granuloma
 Diagnosis : presence of anti proteinase 3 ANCA

54. CHURG-STRAUSS SYNDROME
 Allergic granulomatosis
 Rare multisystem disease characterized by eosinophil-
rich granulomatous inflammation involving the
respiratory tract & necrotizing vasculitis affecting
small to medium sized vessels in association with
asthma & eosinophilitis

55. Clinical features:
 Women – 5th decade
3 phases:
1) 1st phase: by asthma & allergic rhinitis
2) 2nd phase: by peripheral blood eosinophilia &
eosinophilic tissue infiltration, notably by
eosinophilic pneumonia & eosinophilic gastro-
enteritis.
3) 3rd phase: systemic vasculitis, which develops with in
3 yrs of the onset of asthma.

56.  Skin: palpable purpura, large echymosis, livedoreticularis &
infiltrated nodules over scalp
 Fever, anorexia, weakness & weight loss
 Pulmonary haemorrage & glomeulonephritis
 ENT: allergic rhinitis, sinusitis, nasal crusting & nasal polyp.
 CNS: mononeuritis multiplex
 D/D: WEGENERE’S GRANULOMA

57. Microscopic polyangitis
 As systemic , neutrophilic, small vessel vasculitis
without extravascular granuloma & asthma.
 Associated with rapidly progressive renal disease {focal
segmental necrotizing glomerulonephritis} , skin
involvement & antibodies to P ANCA
 Palpable purpuria, petechiae, splinter hemorrhages,
nodules, palmar erythema or livedo-reticularis

58. Criteria:
 Biopsy confirmation of neutrophilic small vessel
vasculitis & or glomerulonephritis with few or no
immune deposits &
 Involvement of more than one organ system
 Surrogate markers such as proteinuria & hematuria for
glomerulonephritis

59. POLYARTERITIS NODOSA
 Classical PAN, periarteritis nodosa
 Characterized by the absence of glomerulonephritis or
vasculitis of arterioles, capillaries or venules.
 Is a segmental vasculitis, obliterative, necrotizing
panarteritis involving small & medium sized muscular
arteries.
 Lesions are at the bifurcations & branching points of
arteries

60. Clinical features
 Common in men, age 45 yrs
 Skin: tender, pulsatile, erythematous nodules present
along the course of blood vessel seen on the lower legs,
dorsum of foot & around the knee.
 Livedoreticularis & digital gangrene are seen.

61.  Constitutional symptoms like weight loss, weakness &
arthralgia
 Systemic: renal failure, HTN, GI Bleeding, infarction,
pancreatic & hepatic infarction, MI, CHF, CVA &
seizures
 SURAL NERVE BIOPSY IS DIAGNOSTIC

62. CUTANEOUS PAN
 Variant of PAN with clinical manifestations primarly
limited to skin.
 Infections such as streptococcus, parvovirus B 19, HIV,
HBV, IBD.
 Seen in all ages, with a predominance in women aged
between 20 & 40 yrs
 Presenting with erythematous, tender nodules over lower
legs, edema, ulcerations, acral gangrene & neuropathy.
 The course of the disease is benign & prolonged.

64.  Severity of C PAN can be classified into 3 groups:
1) Mild disease: nodular skin lesions, livedo reticularis
& neuropathy
2) Severe disease: prominent livedo, ulcers, pain,
sensory neuropathy & constitutional symptoms
3) Progressive systemic disease: constitutional
symptoms, ulcers, acral gangrene, foot drop,
mononeuropathy multiplex, progressive
musculoskeletal involvement & visceral involvement

65. NODULAR VASCULITIS
 Erythema induratum of bazin & of whitfield
 Chronic relapsing lobular panniculitis of subcutaneous
arteries & veins, subsequent ischaemia of
subcutaneous tissue which results in clinical
expression.
 Arising due to a presumptive tubercular origin.

66.  Clinical features: women, age 30 & 60 yrs
 Tender, dusky nodules or plaques  suppurates&
ulcerates over the postero-lateral aspects of legs
 No systemic involvement

68. KAWASAKI DISEASE
 Muco-cutaneous lymph node syndrome
 Is a self limiting, febrile, vasculitic multisystem disease
mainly affecting infants & children < 5 yrs.
 Acute onset of fever, last for 1 wk

69.  Associates with conjunctivitis, cervical
lymphadenopathy, oro-pharyngeal erythema with
denuded, red tongue with prominent papillae –
strawberry tongue & fissured lips.
 Skin: erythema, macular exanthem & edema followed
by desquamation from the fingers & toes

71. GIANT CELL ARTERITIS
 Temporal arteritis
 Systemic disase characterized pathologically by a
granulomatous panarteritis involving large & medium
sized vessels
 Superficial temporal & ophthalmic arteries- common
 Women > 50 yrs

72.  Severe headache, jaw claudication, earache, sorethroat
& constitutional symptoms.
 Classical sign – swollen, tender, pulseless & thick
temporal artery
 Cutaneous are rare-nodules over involved artery.

73. TAKAYASU’S DISEASE
 Aortic arch syndrome, pulseless disease
 Characterized by a chronic inflammatory vasculitis
involving the aorta & its branches.
 Women -2nd or 3rd decades of life
 Initial: constitutional symptoms

74.  Later: pulseless stage, patient may present with bruits,
loss of pulses & ischaemic features in the form of
headache, seizures & angina
 Skin: erythema & ulcerations present

76.  Diagnosis is based on
1) Clinical
2) Histology
3) Imaging
4) Labaratory findings

77.  Confirm diagnosis by biopsy
 Evaluate the patient for the presence & extent of
systemic disease
 Identify any treatable etiology such as drug, infection,
malignancy or systemic disease

78. CLINICAL
LARGE MEDIUM SMALL
-LIMB CLAUDICATION
-ASYMMETRIC BP
-ABSENCE OF PULSES
-AORTIC DILATION
-BRUITS
-CONSTITUTIONAL
SYMPTOMS
-SUBCUTANEOUS NODULES
-ULCERS - DEEP
-LIVEDO-RETICULARIS
-PITTED PALMAR/DIGITAL SCARS
-DIGITAL GANGRENE
-MONONEURITIS
-ANEURYSM
-INFARCT
-ERYTHEMATOUS NODULES
-HYPERTENSION
-CONSTITUTIONAL SYMPTOMS
-PURPURA
-INFILTRATED ERYTHEMA
-URTICARIA
-VESICOBULLOUS LESIONS
-ULCERS-SUPERFICIAL
-SPLINCTER HEMORRHAGE
-SCLERITIS, EPISCLERITIS
-PALISADED NEUTROPHILIC
-GRANULOMATOUS
DERMATITIS
-GLOMERULONEPHRITIS
-GASTRIC COLIC
-PULMONARY HEMORRAGE
-CONSTITUTIONAL
SYMPTOMS
CUTANEOUS LESIONS CORRELATES WITH THE SIZE OF VESSEL WALL

79. FINDINGS SUSPECTED SYSTEMIC VASCULITIC
SYNDROME
CLINICAL SIGNS OR SYMPTOMS
HIGH FEVER INFECTIONS, SYSTEMIC
INFLAMMATORY DISORDERS
PARESTHESIA, FOOT DROP CSS,PAN,
ABDOMINAL PAIN HSP, CSS, MPA, PAN,
FRANK ARTHRITIS INFECTION, PAN
HYPERTENSION PAN
PURPURA ABOVE WAIST, UPPER
EXTREMITIES
HSP, MPA,WG, CSS
>1 TYPE OF VASCULITIC LESION HSP, MPA,WG, CSS
PUNCTATE PALMAR LESION LV

80. HISTOLOGY
 Biopsy has a great impact on the diagnosis
 Optimal time is <48 hrs after the appearance of a
vasculitic lesion.
 Choice of lesion:
- non ulcerated lesion
- tender, reddish or purpuric lesion
- edge of a superficial ulcer
- deep biopsy

81.  Technique :
- shave biopsy
- punch biopsy
- excisional biopsy
- incisional biopsy
 Punch- small & medium vessel vasculitis
 Incisional – large vessel vasculitis
 Deep biopsy extending to the subcutis – significantly
increase the diagnostic yield for vasculitis.

82. Criteria for vasculitis
 Evidence of vessel leakness such as edema &
extravasation of erythrocytes
 Evidence of vessel destruction such as necrosis of
endothelium & deposition of fibrinoid material with
in the vascular lumina or vessel wall.
 Evidence of perivascular inflammatory cell infiltrates
& leukocytoclasis { formation of nuclear dust}.

83. HISTOLOGICAL EXAMINATION
DEEP DERMAL OR SUB CUTANEOUS
SMALL OR MUSCULAR VESEL
VASCULITIS
MPA,WG, CSS, CV,
PALISADED NEUTROPHILIC
GRANULOMATOUS DERMATITIS
WG, CSS, LV,
TISSUE NEUTROPHILIA SLE, INFECTION
TISSUE EOSINOPHILIA CSS & DRUG INDUCED
DIRECT IMMUNOFLORESCENCE
IgA VASCULAR DEPOSITS HSP
LUPUS BAND {IgG, IgM, C3 AT BMZ} LV, UV with SLE

84. Direct immunoflorescence
 Absence of immune complexes
- WG, CSS, MPA
 DEPOSITION OF Ig G, Ig M & or C3 I or around the vessels
- cryoglobulinaemic vasculitis & CTD vasculitis
 IgA vascular deposits
- HSP
 BMZ deposition of IgG
- urticaria vasculitis

85.  CBE
 RFT
 LFT
 CUE
 STOOL
 HBs Ag
 ANCA
 RF
 ANA
 COMPLEMENT LEVELS
 CRYOGLOBULINS
 IMMUNOGLOBULINS
 ECHO
 ASLO
Laboratory work up

86. LABORATORY EVALUATION
ESR > 40 mm /H INFECTION, MALIGNANCIES
ELEVATED RF, CRYOGLOBULINES &
LOW COMPLEMENTS
CV
CHEST X-RAY: INFILTRATES WG, CSS, MPA, MALIGNANCIES
HEMATUIA, PROTEINEURIA,
CREATININE
HSP, MPA,WG, SLE, CV
HYPOCOMPLEMENTEMIA UV associated with SLE,, CV
ABNORMAL BLOOD COUNT INFECTION, MALIGNANCIES,
INFLAMMATORY DISORDERS
C ANCA WG
P- ANCA MPA, CSS
EOSINOPHILIA, ELEVATED IgE CSS

87. Treatment modalities
CATEGORY FINDINGS TREATMENT
GENERAL
MEASURES
MOST CASES OF
CUTANEOUS
VASCULITIS WILL
REPRESENT A SELF-
LIMITED, SHORT
LIVED, SINGLE
EPISODE OF
PURPURA OF THE
LOWER
EXTREMITIES
-CONFIRM DIAGNOSIS
-EXCLUDE SYSTEMIC DISEASE
-TRIGGER OR CAUSATIVE
FACTORS-TREAT/REMOVE
-REST, AVOID STANDING
-LEG ELEVATION
-AVOID COLD EXPOSURE & -
TIGHT FITTING CLOTHING
-ANTIHISTAMINES, ASPIRIN,
NSAIDS
- AVOID SMOKING

88. Specific therapy
ROLE OF NEUTROPHIL-CHEMOTAXIS
INHIBITORS:
 Dapsone-50-200 mg /day in divided doses &
Colchicine -0.6-1.8 mg/day are effective in CSSV.
 In cases of UV, dapsone/ colchicine combined with
indomethacin or HCQS.

89. ROLE OF ANTIMALARIALS:
 HCQS 200-400 mg in UV
ROLE OF CORTICOSTEROIDS:
 In SVV, not responding to dapsone/ colchicine , low
dose steroids- <10 mg on alternate day may be helpful
Indications as 1st line therapy:high dose 60-80 mg/day
 Ulcerative / necrotic cutaneous lesion,
 GI bleeding
 Acute glomerulonephritis
 Peripheral neuropathy

90.  In children with HSP , we can prevent the risk of
developing nephritis
 Patients with Cut-PAN
 Classic PAN
 Vasculitis associated with viral etiology to prevent
replication
 Treat renal & CNS manifestations
 High dose steroids are treatment of option for WG,
MPA & CSS.
 Steroids are contraindicated in kawasaki disease to
avoid the risk of coronary aneurysm formation

91. ROLE OF IMMUNOMODULATORS:
 Wkly dosage of methotrexate, upto 25 mg /wk
Azathioprine, 2mg/kg/day
Cyclophosphamide or cyclosporine in CSSV
 Addition of these drugs with corticosteroids are useful
in treating WG, MPA & CSS with significant systemic
involvement

92. ROLE OF ANTIVIRAL DRUGS:
 In hepatitis C virus associated cases of CV , interferon
alfa - the drug of choice.
 3 million IU of IFN α , thrice wkly, for 12-18 months.
 Ribavirin –to prevent relapses

93. ROLE OF INTRAVENOUS IMMUNOGLOBULINES:
 Treatment of choice for kawasaki disease
 IVIgG -2 gm/kg single dose in combination with
aspirin
 Also useful in HSP & cut PAN.

94. NEWER MODALITIES
 Mycophenolate mofetil is used in WG, MPA & UV
 Infliximab is used in CSS:
 MISCELANEOUS:
 Pencillins- in cut PAN, WG associated streptococcal
infection
 Cotrimoxazole
 Prostaglandin I2 -50 µg/day IV &
 Nifedepine- 60 mg /day
Cut PAN
associated
gangrene

95. Mimics of cutaneous vasculitis:
1) Vascular disorders
 PLC
 PERNIOSIS
2) VASCULAR OCCLUSIVE DISEASE
 DIC
 Thrombocythemia
 Homocystineuria
3) Embolic
 Cholesterol embolism

96. 4) Purpura
 Platelet deficiency
 Drug induced
5) Dermatosis
 Schambergs disease
6) IBR

97. Mimics of systemic
 Drug induced: coccaine, amphetamine
 Infection: SABE
 Embolism
 malignancy

98.  In all cases of vasculitis , the attempt should be to reach a
definite diagnosis rather than using broad, non specific
terminologies.
 Determining prognosis & deciding therapeutic strategy in
these cases depend mostly on the extent of organ
involvement
 Disorders significantly reduced if recognized & treated early.
 Since some of cutaneous involvement is a universal finding
in all types of vasculitis, dermatologists play a crucial role in
diagnosing vasculitic disorders, predicting the extent of
underlying systemic involvement & prognosis