Treatment options for necrobiosis lipoidica: a systematic review

Abstract

BackgroundNecrobiosis lipoidica (NL) is a rare, idiopathic, and recalcitrant disease of collagen degeneration for which treatment options have been poorly studied. Due to its recurring nature, risk for ulceration, and high morbidity, there is a need to understand existing treatment modalities to better inform clinical care.ObjectiveThis review aims to describe the therapeutic modalities reported in the literature for the treatment of NL.MethodsA literature search of treatments was performed by searching for publications between January 2016 and May 2022 on PubMed and Scopus. Given the limited high‐quality evidence, case reports and series were included. Only publications presenting information on both attempted treatments and outcomes were included.ResultsA total of 60 novel articles were identified (54 case reports, two case series, and four retrospective cohort studies). These studies cumulatively reported on 274 patients and covered treatments including phototherapy, topical corticosteroids, topical calcineurin inhibitors, biologics, immunosuppressants, JAK inhibitors, combination therapies, and several others. The greatest amount of evidence was found for photodynamic therapy (improvement in 72 of 80 patients), UVA‐based phototherapy (12 of 33), topical corticosteroids (21 of 46), compression therapy (15 of 20), and topical calcineurin inhibitors (11 of 17). Several newer treatments were also described, including ustekinumab and JAK inhibitors.ConclusionsThis systematic review provides a comprehensive summary of recently published treatments for NL. As the existing data comes predominantly from case reports and series, statistical conclusions are not assessed. A greater number of randomized controlled trials with standardized endpoints are necessary to compare treatment efficacy.

Shedding Light on Photodynamic Therapy in the Treatment of Necrobiosis Lipoidica: A Multicenter Real-Life Experience

“…Limited data on the risk of relapse after discontinuing topical corticosteroid treatment precluded any definitive conclusions. In addition to topical corticosteroids, the most supported treatments for NL include topical calcineurin inhibitors, compression therapy, and classic immunosuppressants and immunomodulators such as methotrexate, cyclosporine, and fumaric acid esters [19]. In a review by Nihal et al, patients treated with topical calcineurin inhibitors showed improvement in 11 out of 17 cases, while compression therapy was effective in 15 out of 20 cases [19].…”

“…Traditional therapies include topical or intralesional corticosteroids, which provide symptomatic relief but may not alter the natural course of the disease [1]. Calcineurin inhibitors and retinoids have been employed as topical alternatives to steroids, with poor results [19]. Systemic therapies, such as immunomodulators and antimalarials, have also been used with varying degrees of success.…”

“…PDT represents a non-invasive, light-based treatment strategy that exploits the interaction between a photosensitizing agent, 5-aminolaevulinic acid (ALA) or its methylated ester (MAL), light, and molecular oxygen to induce localized cytotoxicity. This unique approach has been shown to be effective for various dermatological conditions, including both oncological and infectious ones, and its application in granulomatous disease is gaining attention for its potential to address the underlying inflammatory processes [19]. Regarding this topic, PDT has gained a Strength of Recommendation C and Quality of Evidence III, according to the latest guidelines, for the treatment of NL and granuloma annulare, another granulomatous disease in which PDT has been shown to have similar efficacy as other topical treatments [31,32].…”

A Breakthrough in the Treatment of Necrobiosis Lipoidica? Update on Treatment, Etiopathogenesis, Diagnosis, and Clinical Presentation

“…Biological treatment is used when other therapeutic options are ineffective or there are contraindications to the use of other drugs. Attempts to use biologics have mainly involved TNF-α inhibitors (adalimumab, infliximab, and etanercept) [ 72 , 74 ]. The discovery of a key role for TNF- α in granuloma formation in mouse models provides a theoretical basis for explaining the efficacy of TNF-α inhibitors in granulomatous inflammatory diseases such as NL [ 74 , 75 ].…”

“…Attempts to use biologics have mainly involved TNF-α inhibitors (adalimumab, infliximab, and etanercept) [ 72 , 74 ]. The discovery of a key role for TNF- α in granuloma formation in mouse models provides a theoretical basis for explaining the efficacy of TNF-α inhibitors in granulomatous inflammatory diseases such as NL [ 74 , 75 ]. Although medications in this class have had a beneficial effect on many patients, there were cases reported that did not respond to treatment or the treatment had to be discontinued due to the loss of efficacy or adverse effects [ 76 , 77 , 78 , 79 ].…”