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.

Treatment options for necrobiosis lipoidica: a systematic review.

BACKGROUND: 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. OBJECTIVE: This review aims to describe the therapeutic modalities reported in the literature for the treatment of NL. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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 breast associated with Crohn's disease: a case report.

BACKGROUND: Necrobiosis lipoidica located to the breast; without evidence of glucose intolerance, is extremely rare, and its association to Crohn's disease is not usual. CASE PRESENTATION: We report an interesting case of an association of necrobiosis lipoidica of the breast and Crohn's disease in a 54-year-old Moroccan woman. Skin necrobiotic changes are a characteristic feature in necrobiosis lipoidica, but they are exceptional in metastatic Crohn's disease, since there are only three published cases of necrobiotic skin lesions on the lower leg resembling erythema nodosum in metastatic Crohn's disease. CONCLUSIONS: On the basis of this rare observation, necrobiosis lipoidica without evidence of glucose intolerance should be recognized as a possible cutaneous manifestation or association of Crohn's disease.

Characteristics of ulcerated and non-ulcerated necrobiosis lipoidica.

BACKGROUND: Necrobiosis lipoidica (NL) is complicated by ulceration in up to 35% of cases. METHODS: Retrospective study of patients with NL seen at our institution between January 1, 1992, and May 25, 2021, was conducted. Ulcerated NL (UNL, n = 83) and non-ulcerated NL (NUNL, n = 233) groups were compared. RESULTS: Twenty-six percent (83/316) of patients with NL experienced ulceration. UNL was significantly more likely to be painful (52% vs. 36%, P = 0.01), was more likely to have a lesion-associated cutaneous malignancy (7% vs. 0%, P < 0.001), and had a larger median size (7 vs. 5 cm, P = 0.004) compared to NUNL. Vascular studies were performed on a subset of patients and revealed transcutaneous oxygen pressure (TcPO2) < 40 mm Hg in 53% and venous insufficiency in 62% with no significant differences between UNL and NUNL groups. In patients with unilateral ulceration, mean TcPO2 values (39.7 vs. 46.6 mm Hg), regional perfusion index <0.6 (29% vs. 14%), and TcPO2 < 40 mm Hg (43% vs. 14%) were worse in the ulcerated leg compared to the non-ulcerated leg, but these differences were not statistically significant. CONCLUSIONS: UNL was more likely to be painful, develop lesion-associated malignancy, and be larger in size compared to NUNL. There were no statistically significant differences in venous insufficiency, arterial Doppler/ankle brachial index, or TcPO2 values between UNL and NUNL patients, however, a significant portion of the cohort demonstrated abnormal vascular studies, particularly on TcPO2 and venous insufficiency testing.

Atypical Ulcerative Necrobiosis Lipoidica Diabeticorum: A Case Study.

Necrobiosis lipoidica is a granulomatous skin condition commonly associated with diabetes. When associated with diabetes mellitus, the name expands to necrobiosis lipoidica diabeticorum (NLD). In these patients, this rare condition has an incidence rate of 0.3% to 1.6%. The cause of NLD remains unknown even though many speculations exist in the medical literature. The treatment of this condition has most researchers agreeing on the use of topical steroids for the anti-inflammatory effect on NLD; however, the role of glucose control in the treatment of this disorder has been debatable. The following case presents a patient who, despite better glucose control, did not improve until the introduction of doxycycline.

Ulcerative necrobiosis lipoidica: case report of an atypical presentation and literature review.

Necrobiosis lipoidica (NL) is a rare chronic granulomatous degenerative skin disease by unknown causes, which is mostly associated with diabetes mellitus, usually presenting with typical plaques of the shins. Although less common, some ulcerative forms may be seen in clinical practice. The occurrence of an atypical presentation in one of our patients was the occasion to review the pertinent literature.

Necrobiosis lipoídica: una manifestación más de la diabetes mellitus / Necrobiosis lipoidica: A complication of diabetes mellitus

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Necrobiosis Lipoidica in a Patient with ß-Thalassemia Major: A Case Report and Review of the Literature.

Necrobiosis lipoidica (NL) is a rare granulomatous disease that predominantly affects middle-aged women and is often associated with diabetes mellitus (DM), rheumatoid arthritis (RA) and other metabolic disorders. Thalassemias are the most common hereditary hemoglobin (Hb) disorders worldwide. A few studies investigated dermatologic problems that coexist with ß-thalassemia major (ß-TM). The most common skin disorders in patients with ß-TM are xerosis, urticaria, pseudoxanthoma, hyperpigmentation, leg ulcers and small-vessel vasculitis. Necrobiosis lipoidica has only been occasionally reported in patients with ß-TM. Herein, we present a female with ß-TM and NL. Furthermore, a brief review of the literature was performed.

Necrobiosis lipoidica with rapid response to doxycycline.

Necrobiosis lipoidica (NL) is a rare granulomatous disease of unknown etiology. Multiple therapies may be used with varying efficacy. We report a pediatric patient with a history of type I diabetes mellitus and NL with minimal response to an ultrapotent topical steroid, topical calcineurin inhibitor, and intralesional triamcinolone, complicated by steroid atrophy, who rapidly responded after addition of doxycycline.

Necrobiosis Lipoidica Mimicking Cutaneous Sarcoidosis Finally Treated With an Intralesional Injection of Corticosteroid: A Case Report

BACKGROUND: Necrobiosis lipoidica (NL) and sarcoidosis are granulomatous disorders with an unknown pathogenesis. They may coexist in the same patient, which suggests a possible overlap between these diseases among shared granulomatous inflammatory pathways. Case Presentation: This study presents the case of a non-diabetic 52-year-old woman who presented with red-yellowish border plaques on the face and upper extremities previously diagnosed as sarcoidosis. After 13 years of inappropriate treatment, histopathological findings consistent with the clinical and para-clinical examination suggested the diagnosis of NL. After treatment with an intralesional injection of steroids, significant improvement was observed, and no recurrent lesions were found. CONCLUSION: Necrobiosis lipoidica may mimic cutaneous sarcoidosis. Prompt recognition and treatment of NL can be helpful for managing the disease. J Drugs Dermatol. 2020;19(1):92-94. doi:10.36849/JDD.2020.4675