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Cutaneous Lesions

  1. Small Vessel Vasculitis:
    1. Palpable purpura (m. c manifestation)
    2. Pinpoint papules,
    3. Vesicles (often hemorrhagic)
    4. Pustules
    5. Splinter hemorrhages
    6. Urticaria
    7. EM-like lesions
  1. Medium/Large Vessel Vasculitis:
    1. Painful dermal/subcutaneous nodules       
    2. Ulcers
    3. Livedo reticularis                     
    4. Acrocyanosis
    5. Gangrene                        
    6. Digital infarcts
    7. Papulonecrotic lesions              
    8. Atrophie blanche (atrophic ivory white stellate scars)
  2. Treatment Of Cutaneous Vasculitis
    1. General measures
      1. Avoiding stress, bed rest, elevation of the foot end and keeping extremities warm
      2. Patients with pulmonary involvement, like CSS, are advised to avoid smoking
      3. Antihistamines and NSAIDs [indomethacin] used to reduce symptoms like pruritus and joint pain
      4. Look for triggers: infections, drugs, autoimmune diseases
  3. Specific Therapy
    1. Dapsone [50-200 mg/day] & Colchicine [0.6-1.8 mg/day]
      1. Effective initial agents for treatment of CSVV in the absence of evidence of systemic involvement
      2. M/A– Not known exactly, neutrophil-chemotaxis inhibition
    2. Antimalarials– Hydroxychloroquine (200-400 mg/day) effective in hypocomplementemic urticarial vasculitis
  4. Corticosteroids
    1. In small vessel vasculitis, not responding to dapsone/colchicine, corticosteroid (30-80 mg OD x tapered 2-3 wks) Good control of symptoms
    2. Indications for systemic steroid as first-line therapy include severe ulcerative/necrotic cutaneous lesion, gastrointestinal bleeding, acute glomerulonephritis and peripheral neuropathy with impending paralysis
    3. WG, MPA, PAN and CSS High-dose corticosteroids (1 mg/kg/day) is the initial therapeutic option
    4. Intravenous steroid ‘pulse’ therapy can be administered to patients with life- threatening organ involvement
    5. Systemic corticosteroids Contraindicated in Kawasaki disease to avoid the risk of coronary aneurysm formation
    6. Other immunosuppressive [cytotoxics]can be used for steroid-sparing or additive effect
  5. Immunosuppressive:
    1. Cyclophosphamide– PAN, MPA, WG, CSS, Takayasu’s arteritis
    2. Weekly methotrexate [10-25 mg]
    3. Azathioprine [2 mg/kg/d]
    4. Cyclosporine [5 mg/kg/d]
    5. Melphalan
    6. Chlorambucil
      Role of antiviral drugs
    7. In hepatitis C virus-associated cases of cryoglobulinemic vasculitis, interferon α (IFN α) is the preferred drug
  6. Intravenous Immunoglobulin (IVIG)
    1. IVIg with aspirin is drug of choice for Kawasaki’s disease Prevents future risk of coronary aneurysm formation
    2. Also used in treatment of HSP & cutaneous PAN
    3. Mycophenolate mofetil Used successfully in WG and MPA; Also effective in maintaining remission in HUV
    4. Prostaglandin I2 and nifedipine (60 mg/d) has been tried in cases of c-PAN associated with digital gangrene
    5. Thalidomide– Immunomodulatory, anti-inflammatory & anti-angiogenic drug  Efficacious in the treatment of cutaneous disease in patients with SLE and in Behcet's disease.
Palpable Purpura

1. Vasculitis 2. Infectious emboli
•  Cutaneous small vessel (leukoclastic vasculitis)
•  B.S. Purpura
•  Polyarteritis nodosa
•  Mixed cryoglobulinemia
•  Rheumatic vasculitis
•  Wegener's granulomatosis
•  Microscopic polyangiitis
•  Churg-Straus
•  Acute MI
•  Disseminated gonococcal infection
•  Rocky mountain spotted fever
•  Ecthyma gangrenosum (pseudomonas)
Classification of Panniculitis

Mostly Septal
With vasculitis With vasculitis
•  Leukocytoclastic vasculitis
•  Superficial thrombophlebitis
•  Cutaneous Polyarteritis nodosa
•  Necrobiosis lipidica
•  Scleroderma/Morphea
•  Granuloma annul are (subcutaneous)
•  Rheumatoid nodule
•  Erythema nodosum
•  Necrobiotic xanthogranuloma
Mostly lobular
With vasculitis Without vasculitis
•  Erythema nodosum leprosum (leprosum)
•  Lucio's phenomenon
•  Erythema induratum
•  Crohn disease
•  Nodular vasculitis
•  Neutrophilic lobular panniculitis (associated with RA)
•  Sclerosing panniculitis (Lipodermatosclerosis)
•  Calciphylaxis
•  Oxalosis
•  Sclerema neonaturum
•  Cold panniculitis
•  Lupus panniculitis
•  Subcutaneous fat necrosis of the newborn
•  Post-steroidal panniculitis
•  Dermatomyositis
•  Subcutaneous sarcoidosis
•  Lipoatrophy
•  Traumatic panniculitis
•  Alpha-I antitrypsin deficiency
•  Panniculitis associated with halogenoderma (iodides)

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