Sustained Dapsone-Induced Remission of Hypocomplementemic Urticarial Vasculitis

Abstract

Hypocomplementemic urticarial vasculitis (HUV) is often misdiagnosed. The response to drug therapy may be unsatisfactory, and immunosuppressive therapy may be associated with significant side effects. A 35-year-old patient whose condition was resistant to cyclophosphamide, corticosteroids, pentoxyphylline, cyproheptadine, sulindac, and colchicine was maintained in remission with dapsone, which may be the drug of choice for HUV. Emphysema has been reported to complicate HUV, but this is the first account of vasculitis-related emphysema with no confounding history of tobacco smoke exposure. The relationship of proteolytic injury to the pulmonary and other manifestations is considered, as is the possible role for dapsone in reducing it.

BSACI guideline for the management of chronic urticaria and angioedema

“…Dietary advice-Diets low in salicylates and benzoates have been anecdotally adopted in the management of Dapsone D Several single-case reports of successful treatments of urticarial vasculitis in resistant cases Helped one patient with autoimmune thyroiditis to stop oral steroid treatment [135][136][137] Hydroxy-chloroquine D Improvement of QoL, but no reduction in urticaria scores or medication requirements [138] Methotrexate D Beneficial for corticosteroid-dependent chronic spontaneous urticaria (2 patients) Efficacy in urticarial vasculitis (one patient) [139,140] Stanozolol (Danazol) C Beneficial effects in patients with refractory CIU (with simultaneous cetirizine dose); Long-term effects unknown; drug currently not licensed, but available on a named-patient basis as winstrol, 4 mg in the United Kingdom. Danazol likely to have similar effects [141] Sulfasalazine D Successful in 2 patients with refractory delayed pressure urticaria and angioedema.…”

BSACI guidelines for the management of chronic urticaria and angio‐oedema

“…One was steroid-dependent and managed to come off prednisolone [91,92] Dapsone D Several single case reports of successful treatments of urticarial vasculitis in resistant cases. Helped one patient with autoimmune thyroiditis to stop oral steroid treatment [93,94] Methotrexate D Beneficial for corticosteroid-dependent chronic idiopathic urticaria (two patients). Efficacy in urticarial vasculitis (one patient) [95,96] Stanozolol (Danazol) C Beneficial effects in patients with refractory CIU (with simultaneous cetirizine dose); long-term effects unknown; drug currently not licensed, but available on a named-patient basis as winstrol, 4 mg in the United Kingdom.…”

Urticarial lesions: If not urticaria, what else? The differential diagnosis of urticaria

“…25 Because many patients have relapses after steroid tapering, it is often necessary to proceed to a maintenance treatment and add a steroid-sparing agent. 25 In these cases, dapsone may be the drug of choice, either alone 51,[110][111][112] or in association with other agents 51,113,114 ; in particular, it is effective in patients with a SLE-like presentation of UV. 25 Colchicine 112,115-118 and hydroxychloroquine 119 have also been used with some success.…”