Severe Refractory Erythema Nodosum Leprosum Successfully Treated with the Tumor Necrosis Factor Inhibitor Etanercept

Abstract

Erythema nodosum leprosum (ENL), or type II reaction, is a common complication of lepromatous leprosy that can cause significant patient debility. First-line therapy includes prednisone and thalidomide, with clofazimine reserved for patients who do not respond to first-line treatment. We present the case of a 33-year-old woman with ENL that failed to respond adequately to conventional therapy over a 6-year period. Because of the severe nature of her disease and the adverse effects of therapy that she experienced, a trial of etanercept was undertaken, which led to full resolution of her ENL. The rationale behind our choice of therapy and its future implications are discussed.

Clinical, histopathological, and immunological evaluation of a series of patients with erythema nodosum

“…This may be explained because all of these cytokines are also thought to be produced by neutrophils, which once activated, may in turn activate themselves and other neutrophils in an autocrine or paracrine manner. The findings regarding TNF alpha are in agreement with previous studies suggesting the efficacy of anti‐TNF alpha agents and thalidomide in the treatment of refractory EN . High levels of TNF alpha also explain the high levels of G‐CSF found in our samples.…”

Apremilast in chronic recalcitrant erythema nodosum leprosum: a report of two cases

“…While TNF‐α inhibitors (e.g. infliximab, etanercept) have been used in severe refractory ENL, we could not find any published report on the use of apremilast in such a distressing condition. The advantages of apremilast vs. TNF‐α inhibitors for resource‐poor leprosy‐endemic regions include its much lower cost than biological therapy, ease of administration and lack of a risk of flare‐up of latent tuberculosis.…”

Leprosy in the 21st Century

“…The generation of the cytokines interleukin-2 (IL-2) and gamma interferon by activated CD4 ϩ and CD8 ϩ lymphocytes may explain the effectiveness of thalidomide (35). Based on the inflammatory pattern of cytokine production (59,60), tumor necrosis factor inhibitors may have an effect on treatment of type 2 reactions. Some success has been reported for the use of the drug azathioprine in patients with frequent ENL recurrences (32).…”