Treatment Of Necrobiosis Lipoidica Research Articles

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

Necrobiosis lipoidica (NL) is a rare granulomatous disease of a not fully understood etiopathogenesis. Classically, NL is associated with insulin-dependent diabetes mellitus. The disease often fails to respond to conventional treatments and adversely affects patients' quality of life. First-line medications are usually topical corticosteroids, but patients respond to them with varying degrees of success. Other options include tacrolimus, phototherapy, cyclosporine, fumaric acid esters, and biologics (adalimumab, etanercept, and infliximab). Our review aims to present new therapeutic approaches potentially effective in patients with refractory lesions, describe the presumed etiopathogenesis, and provide diagnostic guidance for clinicians. The review concludes that Janus kinase inhibitors and biologics such as ustekinumab and secukinumab can be used effectively in patients with recalcitrant NL. Another promising treatment option is tapinarof (an aryl hydrocarbon receptor agonist). However, studies on larger groups of patients are still needed to evaluate the effectiveness of different therapeutic options and to define consistent treatment regimens for NL. It is advisable to improve the awareness of physicians of various specialties regarding necrobiosis lipoidica as lesions diagnosed earlier usually have a better response to treatment.

Topical Ruxolitinib in the Treatment of Necrobiosis Lipoidica: A Prospective, Open-Label Study

Necrobiosis Lipoidica (NL) is a rare granulomatous disease. There are few effective treatments for NL. We sought to investigate the efficacy and safety of the JAK 1/2 inhibitor, ruxolitnib, in the treatment of NL and identify biomarkers associated with disease and treatment response. We conducted an open-label, phase 2 study of ruxolitinib in 12 patients with NL. We performed transcriptomic analysis of tissue samples pre- and post-treatment. At week 12, the mean NL lesion score decreased by 58.2% (SD 28.7%, P=0.003). Transcriptomic analysis demonstrated enrichment of type I and type II interferon pathways in baseline disease. Weighted Gene Co-expression Network Analysis (WGCNA) demonstrated post-treatment changes in interferon pathways with key hub genes IFNG and STAT1. Limitations include small sample size and a study group limited to patients with <10% BSA. In conclusion, ruxolitinib is an effective treatment for NL and targets the key pathogenic mediators of disease.

Treatment options for necrobiosis lipoidica: a systematic review.

Necrobiosis 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. This review aims to describe the therapeutic modalities reported in the literature for the treatment of NL. A 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. A 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. This 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.

Necrobiosis lipoidica of the scalp

Necrobiosis lipoidica is a chronic granulomatous skin disease of a vascular-exchange nature from the group of localized skin lipoidosis, accompanied by the development of degenerative changes in the connective tissue.&#x0D; The reason for the development of necrobiosis lipoidica is unknown, but the most common theory remains the theory of vascular disorders, including the deposition of immune complexes (IgM, complement component C3), microangiopathic changes (deposition of a glycoprotein in the walls of blood vessels), as well as other combinations of inflammatory and structural changes leading to collagen degeneration and decreased perfusion and oxygenation of the skin. Necrobiosis lipoidica occurs in association with systemic diseases (sarcoidosis, autoimmune thyroiditis, inflammatory bowel disease, such as ulcerative colitis, and rheumatoid arthritis) and can also occur in otherwise healthy individuals. Necrobiosis lipoidica is closely associated with diabetes mellitus.&#x0D; The predominant localization of necrobiosis lipoidica is tibial surface of the legs. Atypical localization is: skin of the scalp and face.&#x0D; Clinical polymorphism of necrobiosis lipoidica, possibility of transition from one form to another, its combination with other dermatoses can pose difficult diagnostic questions for the doctor.&#x0D; The article describes our own clinical observation of a patient with complaints of rashes on the skin of the legs and scarring alopecia in the scalp. The focus on the scalp had a tendency to grow and signs of development of scarring alopecia. According to the results of a biopsy performed at the Rakhmanov Department of Skin and Veneral Diseases, I.M. Sechenov First Moscow State Medical University, was diagnosed with "Necrobiosis Lipoidica". The case was interesting due to the extreme rarity of rashes on the skin of the scalp and the absence of dermatoscopic features of lesions in this area. Due to ongoing therapy for a month, there was a positive effect in the form of a lack of growth of existing plaques and the appearance of new ones, a decrease in the brightness of the color of the rash, a partial regression with an outcome in residual hyperpigmentation.&#x0D; Thus, despite the absence of a gold standard in the treatment of necrobiosis lipoidica, the most well-studied group of drugs for the treatment of the disease at the moment are glucocorticoids, which confirms our experience. Therapy of patients with necrobiosis lipoidica should be carried out by an interdisciplinary team (dermatovenereologist, endocrinologist, therapist).

LB947 An open-label study of topical ruxolitinib in necrobiosis lipoidica

Necrobiosis lipoidica (NL) is a chronic granulomatous disease of the skin. A growing body of literature suggest that janus kinase (JAK) inhibitors for may be effective in the treatment of granulomatous skin diseases, including NL. Prior translational research identified activation of JAK-STAT pathways by interferon gamma (IFNγ) and other cytokines is integral to macrophage activation in these diseases. We aimed to evaluate the safety and efficacy of topical ruxolitinib, a JAK-1/2 inhibitor, for the treatment of NL.

18226 Laser and light therapies for the treatment of necrobiosis lipoidica

Background: Necrobiosis lipoidica (NL) is a rare inflammatory granulomatous skin disorder involving collagen, elastic, and fiber degeneration. Multiple light and laser therapy modalities have been proposed and used in treatment of NL with variable outcomes and side effects.

Laser and light therapies for the treatment of necrobiosis lipoidica.

Necrobiosis lipoidica (NL) is a rare, inflammatory granulomatous skin disorder involving collagen degeneration. In recent years, several light and laser therapies have been proposed and used in the treatment of NL with variable outcomes. The aim of the study was to investigate the efficacy and safety of lasers and light therapies for the treatment of NL. A review of PubMed was conducted to search for studies using laser and light therapies for the treatment of NL. Articles that employed a combination of treatment modalities were excluded. Twenty-four studies were reviewed. Light and laser therapies used in these studies included CO2 laser, pulsed dye laser, methyl aminolevulinate (MAL)-photodynamic therapy (PDT), aminolevulinic acid (ALA)-PDT, ultraviolet A1 (UVA1) phototherapy, and psoralen plus ultraviolet-A (PUVA). PUVA was identified as the modality with the most available evidence (7 studies), followed by MAL-PDT and ALA-PDT (5 studies each), pulsed dye laser and UVA1 (3 studies each), and lastly CO2 laser (2 studies). Most modalities demonstrated variable efficacies and side effects with the exception of PDL, which consistently showed successful outcomes. Multiple dermatologic light and laser therapies have been investigated for the treatment of NL, including PUVA, ALA-PDT, MAL-PDT, pulsed dye laser, UVA1, and CO2 laser. However, a clear consensus on the preferred treatment is yet to be addressed. Each treatment option demonstrates both advantages and disadvantages that should be discussed with patients when selecting the treatment modality.

Treatment of Necrobiosis Lipoidica in the left forearm with association of Intense Pulsed Light and Erbium-Yag Laser 2940nm

Treatment of Necrobiosis Lipoidica in the left forearm with association of Intense Pulsed Light and Erbium-Yag Laser 2940nm

Study design and preliminary safety and tolerability of PCS499 for the treatment of necrobiosis lipoidica

Study design and preliminary safety and tolerability of PCS499 for the treatment of necrobiosis lipoidica

Unusual presentation and successful treatment of necrobiosis lipoidica in a 15-year-old girl

Necrobiosis lipoidica (NL) is a rare, granulomatous skin disease with a predilection for diabetic patients. NL can present both histopathologically and clinically in a similar fashion to other granulomatous dermatoses such as granuloma annulare, necrobiotic xanthogranuloma, and cutaneous sarcoidosis. When NL is suspected in patients without glucose intolerance, affirmative diagnosis can be difficult, and other comorbidities may be probed, as NL has been associated with conditions such as hypertension, dyslipidemia, and thyroid disorders. Familial disease has also been reported.In the following case report, we discuss a 15-year-old girl who presented with a new, single, pink atrophic plaque of the right pretibial region. Biopsy showed palisaded granulomatous inflammation within the dermis, absent dermal mucin, and rare multinucleated giant cells. This histopathologic description combined with the clinical context led to the diagnosis of necrobiosis lipoidica. The patient developed another similar lesion on the left anterior shin over the next 1.5 years which was also approached as necrobiosis lipoidica. The lesions were treated with intralesional triamcinolone acetonide and topical tacrolimus. Both lesions are now resolved with only mild atrophy of the affected areas. To date, the patient’s labs have shown no evidence of glucose intolerance, hyperlipidemia, or thyroid disorder.

Treatment of necrobiosis lipoidica with intra-lesional glucocorticoid

N/A (Case Report)

Update: Treatment of necrobiosis lipoidica.

Necrobiosis lipoidica (NL) is a rare granulomatous disease of hitherto unclear etiology frequently seen in patients with diabetes. Characterized by its potential for ulcerations, it often presents a serious burden for those affected. There are currently neither German nor European guidelines for the treatment of NL. At the same time, standard treatment with topical or intralesional corticosteroids does not always show satisfactory results. We therefore set out to evaluate whether the various treatment regimens published since 2000 have actually expanded the therapeutic armamentarium in a relevant manner. Included were all publications that described more than one patient being treated with any given therapeutic modality. Overall, we analyzed data for 16 different treatment regimens reported in 49 publications. The largest amount of data exists for topical PUVA therapy, photodynamic therapy (PDT), and systemic treatment with fumaric acid esters. Remarkably, our analysis showed that with an increase in the number of documented patients treated with a given therapeutic modality, the proportion of those achieving a complete or partial response actually decreased. This was interpreted as publication bias. Thus, no clear recommendation can be given for second-line therapy in case topical or intralesional corticosteroids fail.

Necrobiosis lipoidica

Necrobiosis lipoidica and granuloma annulare are granulomatous skin conditions that have been traditionally associated with diabetes mellitus, although recently the strength of association has been questioned. In the first section of this two-part article, we describe the suggested aetiology, clinical features, histology and treatment of necrobiosis lipoidica. It is found in 0.3% of patients with diabetes, but has also been reported with other systemic conditions. Clinically it appears as a waxy, atrophic, yellowish plaque with overlying telangi-ectasia and a brown border. Although usually asymptomatic, it may be extremely painful, especially if ulcerated. Squamous cell carcinoma has occasionally been reported with longstanding lesions. Treatment is often difficult: numerous topical and systemic agents have been employed, but evidence is limited to small case series and individual case reports.

Long-term successful healing of ulcerated necrobiosis lipoidica after topical therapy with becaplermin

We report a case of chronic leg ulceration occurring in a patient with necrobiosis lipoidica (NL). After many topical treatments had failed to achieve healing, treatment with topical becaplermin was started, which resulted in rapid improvement and ultimately complete healing of the ulceration. Treatment of ulcerated NL is often disappointing. Many topical and systemic drugs have been tried. Becaplermin is a platelet-derived growth factor indicated for the treatment of neuropathic diabetic ulcers measuring < 50 mm in size. To our knowledge, this is only the second case reported in the literature of ulcerated NL successfully treated with becaplermin.

Fumaric Acid Esters in Severe Ulcerative Necrobiosis Lipoidica: A Case Report and Evaluation of Current Therapies

Necrobiosis lipoidica (NL) was first described by Oppenheim in 1929. NL is a granulomatous skin disease with association with diabetes mellitus (DM) in 10–40% of cases, but less than 1% of patients with DM develop NL. Additional risk factors for NL include other gra-nulomatous diseases, such as granuloma anulare (GA), sarcoidosis, colitis ulcerosa and Crohn’s disease. Middle-aged women are mainly affected. The lesions typically present as irregular or ovoid plaques with a central yellow atrophic area and a violaceous border; telangiectasia is often present. Ulcerations occur in approximately one-third of patients and complicate the clinical course. The most common sites of NL manifestation are the lower legs. Topical glucocorticosteroids represent the standard treatment of NL. In more complicated cases, glucocortico-steroids are given systemically. Several other therapies, including phototherapy, as well as systemic treatment with antimalarial drugs, cyclosporine and tumour necro-sis factor (TNF) antagonists have been reported (Table I). However, a constantly effective and safe treatment has not yet been established. Especially in patients with steroid-refractory severe ulcerative NL, safe and effective therapies are needed. We report here the first case of a young woman with long-standing ulcerative NL who did not respond to topical and systemic glucocorticosteroids or phototherapy, but was treated successfully with fuma-ric acid esters (FAE).CASE REPORT

Treatment of necrobiosis lipoidica with etanercept and adalimumab

Treatment of necrobiosis lipoidica with etanercept and adalimumab

Response of Ulcerated Necrobiosis Lipoidica to Clofazimine

© 2009 The Authors. doi: 10.2340/00015555-0722 Journal Compilation © 2009 Acta Dermato-Venereologica. ISSN 0001-5555 Sir, Necrobiosis lipoidica (NL) is a chronic granulomatous inflammatory disease of the skin of unknown aetiology, which can be associated with diabetes mellitus. NL is characterized by slowly growing initially erythematous plaques that turn into yellow-brown, partly telangiectatic and atrophic scars. Ulcerations occur in approximately 35% of cases. Many therapies have been recommended for this chronic disease; nevertheless, therapeutic success is rare. We report here the successful treatment of ulcerated NL with clofazimine, without noteworthy side-effects.

Photodynamic Therapy of Necrobiosis Lipoidica – A Multicenter Study of 18 Patients

Background: Necrobiosis lipoidica (NL) is a granulomatous skin disease of unknown origin, and no reliably effective treatment option exists to handle this often disfiguring disease. Recently, a patient with long-lasting NL was reported to be cured by topical photodynamic therapy (PDT). Objective: To evaluate the overall potential of PDT in the treatment of NL on the lower legs. Methods: Retrospective study of 18 patients (aged 16–62 years) from 3 European university departments of dermatology treated with PDT for NL. Methyl aminolevulinate or 5-aminolevulinic acid were used as topically applied photosensitizers. Illumination followed with red light-emitting diode light. Results:Complete response was seen in 1/18 patients after 9 PDT cycles, and partial response in 6/18 patients (2–14 PDT cycles) giving an overall response rate of 39% (7/18). Conclusion: Although almost 40% of the cases showed some degree of response, PDT cannot currently be recommended as first-line therapy of NL. Subpopulations of therapy-resistant NL patients may, however, benefit from PDT.

Treatment of Necrobiosis Lipoidica with 595 nm Pulsed Dye Laser - A Case Report

Introduction: Necrobiosis Lipoidica (NL) had no adequate treatment. Some case has been treated by different vascular lasers. Third generation pulsed dye lasers with a wavelength of 595 nm has been attempted. Their selectively destroys shallower ectatic blood vessels.

Successful Treatment of Necrobiosis Lipoidica With Antimalarial Agents

Successful Treatment of Necrobiosis Lipoidica With Antimalarial Agents