1. CUTANEOUS VASCULITIS
Oktay Taşkapan,MD

2. Vasculitis
A term applied to inflammation and necrosis of blood vessels
Vasculitis may be local or systemic, and may be primary or secondary to another disease process
Small vessels (such as capillaries, arterioles and venules), medium-sized vessels (such as visceral vasculature,including renal, coronary or hepatic arteries) and large vessels (the aorta and its great vessels) may be affected
Many vasculitides have a cutaneous component.

5. Wegener’s granulomatosis
Granulomatosis with polyangiitis (GPA)

7. Small-vessel vasculitides
Small-vessel vasculitides: Henoch–Schönlein purpura (HSP), urticarial vasculitis (UV), cryoglobulinemic vasculitis (CV), and cutaneous small vessel vasculitis (CSVV), commonly referred to as cutaneous leukocytoclastic angiitis
The inflammation involves capillaries, postcapillary venules, and nonmuscular arterioles that reside in the superficial papillary dermis
Palpable purpura is the hallmark cutaneous finding of small-vessel vasculitis
Petechiae, vesicles, pustules,urticaria, and splinter hemorrhages may also be seen

8. Medium-vessel vasculitis
Arterioles with smooth muscle walls that are found within the deeper reticular dermis
PAN and Kawasaki disease (KD) are the predominant medium-vessel vasculitides
Cutaneous findings such as livedo reticularis, subcutaneous nodules, ulcers, digital infarcts, and papulonecrotic lesions are consistent with medium-vessel vasculitis.

9. Mixed-vessel vasculitis
It can involve both small and medium vessels
More diverse cutaneous findings than observed in either small- or medium-vessel vasculitis alone
Microscopic polyangiitis (MPA), Wegener granulomatosis (WG), and Churg–Strauss syndrome (CSS)

10. Large-vessel vasculitis
Takayasu arteritis or giant-cell (temporal) arteritis involve arteries not found within cutaneous tissues
Primarily with nondermatologic findings such as limb or jaw claudication, bruits, pulse defects, and new onset of headache

14. Histopathologic features of cutaneous vasculitis
*Fibrinoid necrosis observed within and around the vessel wall
*Neutrophilic infiltration of the vessel wall, endothelial swelling, granulocytic debris (leukocytoclasia), and extravasated erythrocytes
*Small-vessel vasculitis affects the vasculature in the papillary dermis
*Medium-vessel vasculitis involves vessels in the reticular dermis and subcutaneous layers
* Mixed-vessel vasculitis impacts both sizes of vessels.

15. Dermatologic manifestations of cutaneous vasculitis
Palpable purpura: Symmetrically in dependent regionsPostinflammatory hyperpigmentation
Asymptomatic, pruritic, or cause a burning sensation
Urticarial lesions may also suggest small-vessel vasculitis (UV)
Patients with UV frequently report a burning sensation
Simple urticaria usually starts regressing within 4 hours
Persistence of urticarial lesions for greater than 24 hours raises clinical suspicion of vasculitis
UV usually resolves with residual hyperpigmentation or ecchymoses
Biopsy allows distinction between urticaria and UV

16. Dermatologic manifestations of cutaneous vasculitis
Papulonecrotic lesions are characteristic findings of mixed vasculitides such as WG and CSS
Necrotic papules favor extensor surfaces
Medium-vessel vasculitis: Nodules, ulcers, LR, and digital infarctions
Nodules are typically painful and erythematous and tend to ulcerate

17. Dermatologic manifestations of cutaneous vasculitis
Vasculitic nodules also occur frequently on the lower extremities
Ulcers indicate intense inflammation within the vessel walls
LR occurs when blood flow through muscular arterioles supplying the skin is interrupted

18. Cutaneous manifestations of vasculitis syndromes

19. Pathophysiology of cutaneous vasculitis
Primary (idiopathic) process or the result of a secondary manifestation such as a drug reaction, infection, autoimmune disease, or malignancy
Penicillins, sulfonamides, allopurinol, thiazides, quinolones, propylthiouracil, and hydantoins
Viral hepatitis and subspecies of Neisseria, Rickettsia, and Streptococcus can trigger fibrinoid necrosis
Autoimmune disorders such as connective tissue diseases and systemic vasculitides present with CV
Harbinger for malignancies (lymphoma and leukemia)
Type II and III immune-mediated reactions are the most common pathogenic mechanisms underlying cutaneous vasculitis

20. Diagnostic evaluation of cutaneous vasculitis
A thorough history and physical examination
The history: Chronicity of the symptoms, medications, allergies, family history of cancer, and past medical history including adult-onset asthma, connective tissue disorders, malignancy, and infections
Constitutional symptoms: Fever, night sweats, weight loss, and fatigue
Associated symptoms:Photosensitivity, hemoptysis,recurrent sinusitis, cough, dyspnea, wheezing, abdominal pain, diarrhea, melena, hematuria, arthralgias, paresthesias,muscle weakness, and
testicular pain.

21. The preliminary panel: WBC with cell differential, ESR, serum chemistry profile (blood urea nitrogen, creatinine), liver function tests, blood culture, and urine analysis.
Other relevant tests: Viral hepatitis serologies, ANAs, RF, C3, C4, ANCA, and cryoglobulins
Chest radiographs and WBC are adequate screening tools for malignancy unless other warning signs are present
CT scan may be needed for more thorough examination of the lungs and URT
Skin biopsy provides diagnostic confirmation of CV and should be performed in all suspected cases
Direct immunofluorescence (DIF)

23. Cutaneous Small-Vessel Vasculitis
(cutaneous necrotizing venulitis, leukocytoclasic vasculitis)

24. Cutaneous small-vessel vasculitis
CSVV is vasculitis confined to cutaneous tissues that primarily involves the postcapillary venules
It may occur in the presence of underlying systemic disease or as an idiopathic entity
Commonly associated conditions: Connective tissue diseases (SLE, Sjogren’s syndrome, RA) and infections (viral, bacterial, rickettsial,mycobacterial)
Recurrence occurs in approximately 10% of patients, who typically exhibit arthralgias, cryoglobulinemia, and the absence of fever

26. Cutaneous small-vessel vasculitis
Palpable purpura
Multiple lesions erupt simultaneously and evolve over hours from erythematous macules and papules to palpable purpura
Pustules,hemorrhagic vesicles, or shallow ulcers
Lesions are distributed primarily on areas of dependency, trauma(pathergy), or under tight-fitting garments
It typically spares intertriginous areas
Usually asymptomatic but may burn or itch
Scar formation is rare
Postinflammatory hyperpigmentation is frequently seen

27. Urticarial vasculitis
May be associated with Sjögren syndrome, SLE, and serum sickness
Less frequent associations include infection, drugs, IgM, or IgG gammopathy and hematologic malignancies
Antihistamines are generally less effective than for treating simple urticaria
Most cases of UV respond to corticosteroids
NSAIDs, dapsone, colchicine, hydroxychloroquine, mycophenolate mofetil, and rituximab have been successfully used to treat UV

28. Henoch-Schönlein purpura
The tetrad of palpable purpura, arthritis,bowel angina, and glomerulonephritis
Signs and symptoms of URTI usually precede the onset of HSP by 1 to 2 weeks
Deposition of IgA-containing immune complexes in the small-vessel walls
Most cases of HSP resolve spontaneously
Treatment is largely conservative
Symptomatic relief of arthritis can be achieved with NSAIDs
Corticosteroids may be useful for treating arthritis and gastrointestinal (GI) symptoms

29. Wegener’s granulomatosis
Cutaneous signs are present in 46 to 66% of cases during the generalized phase: Palpable purpura, oropharyngeal ulceration
These ulcers have violaceous, corrugated borders that resemble pyoderma gangrenosum
Papulonecrotic lesions may be present and usually favor the extremities (especially the elbows) but also occur on the face and scalp
Subcutaneous nodules, papules, vesicles, and splinter hemorrhages have also been observed

30. Churg-Strauss syndrome
Cutaneous lesions are seen in 40 to 70% of patients with palpable purpura being present in nearly 50% of cases
Subcutaneous nodules are observed in 30% of patients and commonly located on the scalp and limbs
Papulonecrotic lesions
LR and cutaneous infarction also occur

32. Cutaneous small-vessel vasculitis
The key factors to consider in diagnosing and treating
CSVV are (1) excluding systemic involvement and (2)
identifying and removing the offending agent
For cases associated with an underlying disease process or infection,appropriate targeted therapy is recommended
Conservative therapies such as loose-fitting clothing,
protection from cold temperatures and sunlight, activity reduction, and extremity elevation

33. Epilogue
Cutaneous vasculitis presents in a wide variety of conditions with different degrees of systemic involvement
It is crucial to obtain a careful history, physical examination,and necessary diagnostic tests to distinguish self-limited conditions confined to the skin from those with systemic involvement and serious sequelae
Patients with cutaneous vasculitis typically follow 1 of 3 types of clinical courses in order of decreasing incidence: (1) a singular, self-limited episode triggered by prolonged exercise, allergy, drug, chemical, or infectious agent; (2) a relapsing, remitting cycle; or (3) a chronic, progressive course
Close interaction between the rheumatologist and the dermatologist may be critical in analyzing the fine details. Familiarity with the key clinical details of these various systemic illnesses is much more important than the serologic tests.