Necrobiosis Lipoidica Diabeticorum

Scaramuzza, Andrea; Macedoni, Maddalena; Tadini, G; Angelis, Laura De; Redaelli, Francesca; Gazzarri, Alessandra; Comaschi, Valentina; Giani, Elisa; Zuccotti, Gian Vincenzo

doi:10.1155/2012/152602

Cited by 4 publications

(5 citation statements)

References 11 publications

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“…There were 27 case reports and two case series that included 31 cases in total (Table 1). 6,11–38 No prospective or retrospective studies were included as none were detected in the search of literature. All available details, such as the exact length of treatment or dosage of medication, are listed in Table 1; most patients received multiple therapies.…”

Section: Resultsmentioning

confidence: 99%

Necrobiosis lipoidica in childhood: a review of literature with emphasis on therapy

Schiefer‐Niederkorn,

Sadoghi,

Binder

2023

J Deutsche Derma Gesell

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SummaryNecrobiosis lipoidica (NL) is a rare chronic granulomatous disease that manifests as sharply demarcated, telangiectatic, brownish‐red plaques with atrophic yellowish centers prone to ulceration and occurs predominantly on the shins. In children, NL is extremely rare, but resistance to therapy, troublesome cosmetic appearance, painful ulcerations, and possible development of squamous cell carcinoma in long‐persisting lesions are challenges during treatment. Our review includes 29 reports of NL in patients aged <18 years published from 1990 on PubMed, EMBASE, and Medline. The mean age of patients was 14.3 years, with a female predominance of 2 : 1 and a high prevalence of diabetes mellitus (80%). Data showed that potent topical steroids up to twice daily is the first‐line treatment. For refractory cases, therapy can be switched to tacrolimus. Ulcerations benefit from phase‐adapted wound care and anti‐inflammatory medical dressings such as medical honey. Adding hyperbaric oxygenation to local or systemic therapy in difficult‐to‐treat ulcerated lesions can be considered. Refractory cases may be switched to topical photochemotherapy or systemic treatment with TNF‐α inhibitors, systemic steroids (preferably in non‐diabetic patients), pentoxifylline, or hydroxychloroquine. Necrobiosis lipoidica in childhood is difficult to treat, with a treatment failure rate of 40%. Therefore, further research through patient registries is recommended.

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Section: Resultsmentioning

confidence: 99%

Necrobiosis lipoidica in childhood: a review of literature with emphasis on therapy

Schiefer‐Niederkorn,

Sadoghi,

Binder

2023

J Deutsche Derma Gesell

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“…Immune-complex vasculitis and collagen abnormalities may also represent potential underlying causes. 4 Previous studies have shown that glucose control does not appear to be correlated with NL onset and severity. However, a study by Cohen concluded on the contrary, that NL is usually associated with poor glucose control and that a tighter glucose control, as currently practiced, might improve or prevent this disorder.…”

Section: Discussionmentioning

confidence: 95%

“…However, due to the significant relationship with diabetes (75–90% of patients with NL have or will develop T1DM), many studies have proposed microangiopathy as leading etiological suspect. Immune-complex vasculitis and collagen abnormalities may also represent potential underlying causes 4 . Previous studies have shown that glucose control does not appear to be correlated with NL onset and severity.…”

Section: Discussionmentioning

confidence: 99%

Necrobiosis Lipoidica Diabeticorum: A pediatric case report

Bonura

Frontino

Rigamonti

et al. 2014

Dermato-Endocrinology

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Introduction: Necrobiosis lipoidica (NL) is a rare chronic granulomatous dermatitis that usually appears in the lower extremities. It affects about 0.3–1.2% of diabetic patients, the majority of whom have type 1 diabetes. The etiology and pathogenesis of this disorder are still unclear. NL is characterized by skin rash that usually affects the shins. The average onset is 30 years, with females being affected more commonly. There are very few reported cases of necrobiosis lipoidica in children. Case report: We report a case of a 16 year old girl affected by type 1 diabetes mellitus (15 years disease duration) who developed an erythematous nodular rash on the lower extremities and interscapular area. In the suspect of necrobiosis lipoidica, a skin biopsy was performed (lower extremities and interscapular area). The microscopic evaluation of the pretibial lesions was suggestive of necrobiosis lipoidica. The smaller lesions in the interscapular area showed signs of perivascular dermatitis which could be consistent with early stages of necrobiosis lipoidica. Local treatment with tacrolimus determined a progressive improvement of the lesions. Conclusion: In patients with T1DM, diagnosis of NL of the lower legs is usually unequivocal. However, diagnosis may be more challenging in the presence of lesions with recent onset and/or atypical clinical presentation and unusual site. In these cases, NL must always be taken in consideration in order to avoid misdiagnosis, wrong/late treatment decisions and progression to ulceration.

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“…Unusual cases of lesions on the face, scalp, trunk, penis, and upper extremities have been reported [ 1 ]. Unusual localizations have also been described in pediatric patients, with lesions observed in the interscapular region, on the abdomen, and on the extensor surface of the upper arm [ 3 , 4 , 37 , 38 ]. In NL, Köbner’s sign may occur, and lesions appear in surgical scars and after skin trauma [ 9 , 39 ].…”

Section: Clinical Featuresmentioning

confidence: 99%

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

Naumowicz,

Modzelewski,

Macko

et al. 2024

IJMS

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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.

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Necrobiosis Lipoidica Diabeticorum

Cited by 4 publications

References 11 publications

Necrobiosis lipoidica in childhood: a review of literature with emphasis on therapy

Necrobiosis lipoidica in childhood: a review of literature with emphasis on therapy

Necrobiosis Lipoidica Diabeticorum: A pediatric case report

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

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