Case Report: Rapid Response to Low-Dose Thalidomide in a Case of Severe Steroid Recalcitrant Erythema Nodosum Leprosum

Abstract

Erythema nodosum leprosum (ENL), or type 2 lepra reaction, presents with crops of evanescent, tender erythematous nodules accompanied by fever, arthralgia, weight loss, malaise, and organ-specific manifestations, and is seen in borderline and lepromatous leprosy. The drugs approved for ENL include nonsteroidal anti-inflammatory drugs, systemic steroids, thalidomide, and clofazimine. The management of ENL is challenging because long-term steroid use leads to steroid dependence. Our patient had severe steroid recalcitrant ENL with vesicular and pustular lesions mimicking Sweet’s syndrome, and was treated effectively with a low-dose thalidomide regimen (100 mg/d) as opposed to the high dose (400 mg/d) recommended in the literature. We discuss the patho-mechanics and clinical utility of a low-dose thalidomide regimen as an effective treatment option for ENL.

Refractory angiolymphoid hyperplasia with eosinophilia: Complete resolution with low dose thalidomide

“…3,4 Peripheral neuropathy is more likely at higher doses, and with cumulative doses >40 g. Thus, lower doses should be considered if long-term use is likely. 5 We report the successful use of low-dose thalidomide in an adolescent with ALHE, previously refractory to multiple treatment modalities.…”

“…Peripheral neuropathy is an uncommon adverse effect with dermatologic use of thalidomide; sedation, drowsiness, and constipation being more typical side effects 3,4 . Peripheral neuropathy is more likely at higher doses, and with cumulative doses >40 g. Thus, lower doses should be considered if long‐term use is likely 5 …”

Update on pharmacology, actions, dosimetry and regimens of oral glucocorticoids in dermatology

“…Any drug that has an additive or synergistic effect in suppressing the disease activity, thus enabling reduction in the glucocorticoid dose, is used as a “glucocorticoid‐sparing agent.” 3,71 There are numerous agents that can be used and these can be used to replace or supplant the relevant action of steroids in various steroid responsive disease. They include colchicine 72 & thalidomide (ENL), 73 Azathioprine (pemphigus), 74 Clofazimine (orofacial granulomatosis), 75 minocycline, leflunomide(alopecia areata), 76 and SSKI (panniculitis), 77 which are a few among the myriad other drugs that can be used in conjunction or as a replacement to steroids.…”

A real‐world study of low‐dose thalidomide in severe erythema nodosum leprosum highlighting its mechanistic rationale in a resource‐constrained target population

“…3 This entails the use of 25 mg to 150 mg/day of thalidomide. 4 The proposed mechanism of action of the drug in ENL, includes its action on interleukin (IL)-12, IL-1b, and neutrophils though the effect on cytokines is variable and possibly its rapid effect is mediated by the action on neutrophils.…”

“…Another rarely employed dosimetry is low‐dose thalidomide, which may be as beneficial as the high‐dose regimen, allowing for the usage of the drug in resource‐constrained settings as well 3 . This entails the use of 25 mg to 150 mg/day of thalidomide 4 . The proposed mechanism of action of the drug in ENL, includes its action on interleukin (IL)‐12, IL‐1β, and neutrophils though the effect on cytokines is variable and possibly its rapid effect is mediated by the action on neutrophils.…”