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CUTANEOUS VASCULITIS IHAB YOUNIS,MD. Classification. I- Small Vessel vasculitis 1- Cutaneous vasculitis 2- Henoch-Schoelein purpura 3- Urticarial vasculitis 4- Erythema elevatum diutinum 5-Granuloma faciale 6-Erythema induratum(Nodular vasculitis)
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CUTANEOUS VASCULITIS IHAB YOUNIS,MD
Classification I- Small Vessel vasculitis 1- Cutaneous vasculitis 2- Henoch-Schoelein purpura 3- Urticarial vasculitis 4- Erythema elevatum diutinum 5-Granuloma faciale 6-Erythema induratum(Nodular vasculitis) II- Large vessel vasculitis Polyarteritis nodosa
III- Neutrophilic vascular reactions: 1- Sweet’s syndrome 2 - Behcet’s disease 3 - Erythema nodosum 4- Pyoderma gangrenosum IV- Overlap: Pityriasis lichenoides chronica
Etiology • The exact mechanisms remain unknown • Although immune complexes are involved in the pathogenesis, other autoantibodies such as antineutrophil cytoplasmic antibody, other inflammatory mediators and local factors that involve the endothelial cells and adhesion molecules play an important role
1-Between one third and one half of the cases of cutaneous vasculitis are idiopathic 2-Drugs: almost all drugs are potential causes . However, The most common drugs are antibiotics, particularly beta-lactam drugs, nonsteroidal anti-inflammatory drugs, diuretics and foreign proteins such as streptokinase and those found in vaccines
3-Various infections may be associated: • Upper respiratory tract infections and viral hepatitis are implicated most often
3-Foods or food additives may cause vasculitis. 4-Collagen vascular diseases ( rheumatoid arthritis, Sjögren syndrome & SLE in particular) account for 10-15% of vasculitis cases 5- Malignancy accounts for less than 1% of cases of cutaneous vasculitis
Extravasation of erythrocytes Perivascular infiltration of polymos with formation of nuclear dust (leukocytoclasis) Fibrinoid necrosisof vessel walls
Clinically • Palpable purpura is the most frequent: • Lesions usually are round and 1-3 mm. • They may coalesce to form plaques, and in some instances, they may ulcerate • Observed most often on the legs, but any surface can be involved • Ecchymosis may be seen
Vesicles &hemorrhagic bullae and in severe cases necrotic areas & ulceration occur • Patients may also experience systemic symptoms with fever, joint, pains and stomach upsets at any time during an attack • The acute rash usually subsides within 2-3 weeks but may recur
Subacute form
Associations • Renal • Joint • Gastrointestinal • CNS • Pulmonary
Investigations • It serves 2 purposes: The first is determining the presence of systemic disease. The second is identifying an associated disorder, which aids in the prediction of the patient's prognosis. • Although no routine has been established, testing for all adult patients includes complete blood cell count, erythrocyte sedimentation rate, urinalysis and blood chemistry panel
Serologic studies, such as antinuclear antibody, ANCA, and rheumatoid factor, should be obtained in patients without an obvious cause of their disease • In patients suggested to have lupus erythematosus or patients who have urticarial vasculitis, complement levels may be obtained
In patients without otherwise identified disease, tests for paraproteins should include serum protein electrophoresis, cryoglobulins, and hepatitis C antibody • Chest radiograph is part of the routine evaluation
Treatment • Elevation of the legs or compression stockings may be useful • Patients with an identifiable cause should receive treatment for that cause e.g. removal of a drug thought to be causing the vasculitis may result in rapid clearing of the process in as short as 2 weeks • Patients with urticarial lesions may be treated with antihistamines, sometimes, a combination of these agents is needed to control the disease • Some patients respond to nonsteroidal anti-inflammatory agents
Anti-inflammatory agents: 1- Colchicine (Colmetidine) 0.5-0.6 mg PO bid/tid Has effects against neutrophils -Adverse effects: Pregnancy category D , risk of renal failure, hepatic failure, permanent hair loss, bone marrow suppression, numbness or tingling in hands and feet, disseminated intravascular coagulopathy and decreased sperm count may occur; dose-dependent GI upset is common
2-Dapsone: 150-200 PO qd(once/day) Precautions -Perform weekly blood counts (first mo), then perform WBC counts monthly (6 mo), then semiannually; discontinue if significant reduction in platelets, WBC, or RBC counts is seen - Caution in G-6-PD deficiency (patients receiving >200 mg/d)
In severecases or if visceral involvement is found, patients may require high doses of corticosteroids (1-2 mg/kg/d) • In refractory cases, an immunosuppressive agent (eg,cyclophosphamide 1-2 mg/Kg/day, azathioprine 1-3 mg/Kg/d, methotrexate 10-25 mg/ week)
2-Henoch-Schonlein Purpura
It was first described by Schönlein in 1837 and the manifestations of abdominal pain and nephritis were reported by Henoch in 1874 • The peak prevalence of HSP is in children aged 3-10 years • In the Northern Hemisphere, the disease occurs mostly from November to January
Etiology • The etiology of HSP remains unknown • However, IgA clearly plays a critical role in the immunopathogenesis of HSP, as evidenced by increased serum IgA , IgA-containing circulating immune complexes and IgA deposition in vessel walls resulting in elaboration of inflammatory mediators, including vascular prostaglandins such as prostacyclin
An antigen may stimulates the production of IgA. Allergens may include: - Foods -Insect bites - Cold exposure - Drugs (eg, ampicillin, erythromycin, penicillin) - Infections (eg, Haemophilus,Mycoplasma, Shigella, Salmonell,adenoviruses, Epstein-Barr virus,parvoviruses,varicella) -Vaccines such as cholera, measles
Clinically • HSP begins with a symmetrical erythematous macular rash on the lower extremities that quickly evolves into palpable purpura • The rash can initially be confined to malleolar skin, but it usually extends to the dorsal surface of the legs, the buttocks, and the ulnar side of the arms
Within 12-24 hours, the macules evolve into palpable purpuric lesions that are dusky red and have a diameter of 0.5-2 cm • The lesions may coalesce into larger plaques that resemble ecchymoses
GI manifestations occur in about 50% of cases and usually consist of colicky abdominal pain, melena, or bloody diarrhea. Hematemesis occurs less frequently • Arthralgia occurs in 60-84% of cases, commonly affecting the knees and ankles
The most serious complication of HSP is renal involvement. It occurs in 50% of older children but is only serious in approximately 10% of patients • Heart: Vasculitis involving the myocardia can occu. • Lungs: Vasculitis involving the lungs can occur, resulting in pulmonary hemorrhage
Investigations • No specific diagnostic laboratory markers exist • The plasma coagulation factor XIII is reduced in about 50% of patients • Urinalysis reveals hematuria. Proteinuria may also be found • CBC can show leukocytosis with eosinophilia and a left shift. Thrombocytosis is present in 67% of cases • Serum IgA levels are increased in about 50% of patients during the acute phase of illness • The antistreptolysin O (ASO) titer is elevated in 30% of cases
Imaging Studies: • Abdominal ultrasonography can be used if GI symptoms are present • Chest radiography may help to determine the presence and extent of pulmonary hemorrhage
To date, no form of therapy has been shown to appreciably shorten the duration of HSP; thus, treatment for most patients remains primarily supportive • Corticosteroids may be considered for the following serious situations: - Persistent nephrotic syndrome -Crescents in more than 50% of glomeruli -Severe abdominal pain -Substantial GI hemorrhage -Severe soft tissue edema -Severe scrotal edema -Neurologic system involvement -Intrapulmonary hemorrhage
The following protocol is suggested: • Induction with 250-750 mg of IV methylprednisolone every day for 3-7 days plus cyclophosphamide 100-200 mg/d PO • Maintenance with prednisone 100-200 mg PO every other day, plus cyclophosphamide 100-200 mg/d PO 30-75 days • Taper prednisone by approximately 25 mg/mo, while the cyclophosphamide dose remains constant • Discontinue treatment after at least 6 mo by abruptly discontinuing cyclophosphamide and complete prednisone taper
Etiology • It is strongly associated with connective tissue diseases e.g. it is present in 20 % of patients with SLE • Other associations include: physical urticarias, hepatitis, cance colon or drugs as AC inhibitors, fluoxetine, thiazides • Immune complexes(circulating and deposited in vessel walls) can be found in 75% of cases
Clinically • Erythematous, indurated weals with purpuric foci • Weals tend to be of longer duration, often longer than 24 hours and tend to resolve with some residual pigmentation or ecchymosis. Patients experience more burning rather than itching
Angioedema,livedoreticularis, nodules and bullae may be found • Constitutional symptoms, GIT & respiratory symptoms, lymphadeopathy, glomerulonephritis & occular affection may be present
Investigations • ESR is elevated • Look for hypocomplementemia • Look for changes of associated signs
Treatment • Corticosteroids : 0.5-1.5 mg/kg/d PO initial; taper as disease responds; if chronic use required, qod administration is safer • Other drugs that are effective include: - Dapsone 100-200 mg once daily - Colchicine 0.6mg twice daiy - Hydroxychloroquine (Alexoquine 250 mg) 200 mg once or twice daily • Oral antihistamines for angioedema
Etiology • First described in 1888 by Hutchinson and in 1889 by Bury • The pathophysiology of EED is not well understood • Direct immunofluorescence shows deposits of complement as well as IgG, IgM, IgA and fibrin around the damaged vessels • Initiation of EED may occur via activation of cytokines such as interleukin 8, which causes selective recruitment of leukocytes to tissue sites
Clinically • Patients usually present with persistent, firm lesions on the extensor surfaces of their skin, especially over the joints • These lesions are most often nodules and round or oval plaques • The color of the lesions progresses over time from yellow or pinkish to red, purple, or brown • Lesions increase in number and size over time. They may also enlarge during the day and return to their previous size overnight
The lesions can be completely asymptomatic, painful, or cause a sensation of burning or itching. These symptoms can be exacerbated by cold • The lesions usually feel firm and are freely movable over the underlying tissue
Investigations • The erythrocyte sedimentation rate in patients with EED is often elevated • Direct immunofluorescence study results show including deposits of complement, immunoglobulins (IgG, IgA, IgM) and fibrin intravascularly and perivascularly