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Chapter 128: Cutaneous Necrotizing Venulitis

Nicholas A. Soter

CUTANEOUS NECROTIZING VENULITIS

Therapeutic Approach

Cutaneous necrotizing vasculitis predominantly involves venules and is known as cutaneous necrotizing vasculitis/venulitis (CNV) and leukocytoclastic vasculitis. CNV maybe restricted to the skin, may occur in association with underlying chronic diseases, may be precipitated by infections or drugs, or may be idiopathic. Idiopathic disorders include IgA vasculitis (IgAV), urticarial vasculitis (UV), and idiopathic vasculitis (IV). Therapeutic approaches will be limited to these idiopathic disorders. Treatment often is unnecessary since the disease may be self-limited with spontaneous resolution, and the focus is on the alleviation of symptoms. Often combinations of therapies rather than a single agent are used, which make it difficult to evaluate the effect of therapeutic agents in the treatment. In the literature, treatment is based on case reports and small case series. None of these agents are FDA-approved. In the author’s experience, treatment usually is initiated with indomethacin and colchicine followed by dapsone and mycophenolate mofetil. The other agents are rarely used and the author almost never uses prednisone. See Table 128-1.

Table 128-1Cutaneous Necrotizing Venulitis Treatment Table
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Table 128-1 Cutaneous Necrotizing Venulitis Treatment Table
MEDICATION NAME INDICATIONa MECHANISM OF ACTION DOSING ADVERSE EFFECTS SUGGESTED MONITORING LEVEL OF EVIDENCE
Indomethacin IgAV, UV, IV Anti-inflammatory 25-50 mg TID Gastrointestinal toxicity None III1-4
Colchicine IgAV, UV, IV Microtubule destabilization, antimitotic, anti-inflammatory 0.6 mg BID or TID Abdominal discomfort None IB5-7
Dapsone IgAV, UV, IV Anti-inflammatory 50-200 mg QD Hematologic toxicity, dapsone hypersensitivity syndrome, haemolysis CBC, CMP, reticulocyte count with each increase in dose, then every 3 mo, check G6PD status before starting dapsone III7-9
Prednisone IgAV, UV, IV Anti-inflammatory, immunosuppression 0.5-1 mg/kg QD Hypertension, hyperlipidemia, myopathy, mood and cognitive effects, increased risk of infection, cataracts, adrenal suppression, diabetes mellitus, gastrointestinal bleeding, peptic ulcer disease, osteoporosis CBC, CMP every 3 mo, monitor blood pressure each visit, ophthalmologic evaluation yearly, absorptiometry (DEXA) scan yearly III2-4,7,10
Cyclosporin IgAV, UV, IV Anti-inflammatory, immunomodulatory 3-5 mg/kg QD Gastrointestinal toxicity, hyperglycemia, abnormal liver and renal function, hypertension, neurologic effects CBC, CMP monthly, monitor blood pressure each visit III2,7,11
Methotrexate IgAV, UV, IV Immunosuppression 25-50 mg weekly Hepatoxicity CBC, CMP, hepatic evaluation with each increase in dose up to 25 mg, then after each 1.5 g dose III7
Mycophenolate mofetil IgAV, UV, IV Immunosuppression 2-3 g QD Gastrointestinal toxicity CBC, CMP with each increase dose, then monthly III7,12
Azathioprine IgAV, UV, IV Anti-inflammatory, immunosuppression 1-2 mg/kg QD Leukopenia, bone-marrow suppression, gastrointestinal toxicity, hepato toxicity CBC, CMP after 2 wk, then monthly, check TPMT enzyme activity before starting treatment III7,13,14
Cyclophosphamide IgAV, UV, IV Immunosuppression 1-2 mg/kg QD Leukopenia, gastrointestinal toxicity, thrombocytopenia purpura, infection CBS, CMP after 2 wk, then monthly IV15
Hydroxychloroquine UV Antimalarial 200-400 mg QD Ophthalmologic toxicity Ophthalmologic evaluation ...

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