Presentation
This article reviews the etiopathogenesis, clinical presentations, and evidence for treatment alternatives of this condition. [dialnet.unirioja.es]
She had no systemic symptoms or complaints at the time of presentation. [karger.com]
Review the presentation of necrobiosis lipoidica. Summarize the treatment of necrobiosis lipoidica. [ncbi.nlm.nih.gov]
Citation: Endocrinology, Diabetes & Metabolism Case Reports 2022, 1; 10.1530/EDM-21-0185 Download Figure Case presentation #4 A 22-year-old primiparous woman presented for the management of T1DM in the context of unplanned pregnancy. [edm.bioscientifica.com]
When a lesion is in its early stages, neutrophils may be present, whereas in later stages of development lymphocytes and histiocytes may be more predominant. [en.wikipedia.org]
Entire Body System
- Inflammation
In addition, “layered” tiers of granulomatous inflammation are composed of histiocytes surrounding horizontal tiers of degenerated collagen. A biopsy from areas of sclerosis can be done, and focal loss of elastic tissue may be seen. [karger.com]
Investigation #4 A shave biopsy of the left-sided plaque demonstrated perivascular inflammation and altered collagen in the upper layers of the reticular dermis. Histopathological findings were consistent with NL. [edm.bioscientifica.com]
It is thought to be linked to blood vessel inflammation related to autoimmune factors. This damages proteins in the skin (collagen). People with type 1 diabetes are more likely to get NLD than those with type 2 diabetes. [ucsfhealth.org]
[…] one or a combination of the following treatments depending on the severity of the symptoms: oral corticosteroids, such as prednisone corticosteroid injections into the inflamed areas of skin antimalarial drugs, such as hydroxychloroquine, to reduce inflammation [medicalnewstoday.com]
- Asymptomatic
The lesions are commonly asymptomatic, but affected skin areas may be fragile, and painful ulcerations develop in many cases. In rare cases, development of squamous cell carcinoma within longstanding lesions has been reported. [orpha.net]
The patches: Most often occur on both shins and are found in other sites only rarely May be asymptomatic or tender May be round, oval, or an irregular shape Central atrophy - shiny, pale, and thinned, with prominent blood vessels (telangiectasia) May [dermnetnz.org]
The lesions are often asymptomatic but may become tender and ulcerate when injured. The first symptom of NL is often a "bruised" appearance (erythema) that is not necessarily associated with a known injury. [en.wikipedia.org]
Our patient had a solitary asymptomatic enlarging NL plaque over her arm that, to our knowledge, is the first in the literature. [karger.com]
While most remains relatively asymptomatic, pruritus, dysesthesia, and pain can occur. Pain is often intense in the presence of ulcerated plaques. [edm.bioscientifica.com]
Skin
- Dermatitis
It was first described by Oppenheim1 under the title of dermatitis atrophicans lipoides diabetica, but most of the reported cases have been under the title of necrobiosis lipoidica diabeticorum, as the entity was named by Urbach.2 This precedent was followed [jamanetwork.com]
[…] autoimmune Keyword (en) Association Chemotherapy Corticosteroid Necrobiosis lipoidica Insulin dependent diabetes Case study Female Human Treatment Endocrinopathy Immunopathology Autoimmune disease Keyword (es) Asociación Quimioterapia Corticoesteroide Dermatitis [pascal-francis.inist.fr]
Necrobiosis lipoidica was first mentioned as dermatitis atrophicans lipoidica diabetica in 1929, by Oppehhein. However, in 1932, Urbach renamed the disease necrobiosis lipoidica diabeticorum (NLD). [pubmed.ncbi.nlm.nih.gov]
Moreover, if necrobiosis lipoidica becomes chronic, it may rarely turn into squamous cell carcinoma.[1][2][3] Necrobiosis lipoidica was first mentioned as dermatitis atrophicans lipoidica diabetica in 1929 by Oppenheim. [ncbi.nlm.nih.gov]
A biopsy from the palpable inflammatory border can be performed, and a diffuse palisaded and interstitial granulomatous dermatitis that involves the entire dermis and extends into the subcutaneous fat septae is usually seen. [karger.com]
- Skin Disease
Homepage Rare diseases Search Search for a rare disease Disease definition A rare skin disease characterized by enlarging, annular plaques with red-brown edges and atrophic, yellow-brown, telangiectatic centers. [orpha.net]
FDA-regulated Device Product: No Additional relevant MeSH terms: Layout table for MeSH terms Necrobiotic Disorders Necrobiosis Lipoidica Collagen Diseases Connective Tissue Diseases Skin Diseases Skin Diseases, Metabolic Metabolic Diseases [clinicaltrials.gov]
Non-infectious granulomatous diseases of the skin and their associated systemic diseases: an evidence-based update to important clinical questions. Am J Clin Dermatol. 2010;11(3):171-81. [PubMed: 20184390] 19. [ncbi.nlm.nih.gov]
- Erythema
The first symptom of NL is often a "bruised" appearance (erythema) that is not necessarily associated with a known injury. The extent to which NL is inherited is unknown. NLD appears as a hardened, raised area of the skin. [en.wikipedia.org]
Dermatological opinion was sought, and a differential diagnosis of granuloma annulare or erythema nodosum was proposed. [edm.bioscientifica.com]
- Xanthoma
[…] presentations can be hard to recognize.[26] The following are some important considerations: Acute complications of sarcoidosis Granuloma annulare Hematologic malignancies Paraproteinemia Rheumatoid arthritis Sarcoidosis Ulcerated necrobiotic Xanthogranuloma Xanthomas [ncbi.nlm.nih.gov]
Workup
Workup in necrobiosis lipoidica At low magnification, lesions of necrobiosis lipoidica have a very characteristic appearance. The granulomas are arranged in a tierlike (layered) fashion and are admixed with areas of collagen degeneration. [emedicine.medscape.com]
Treatment
Currently no FDA-approved treatment exists, and no well-established treatment algorithm has been described. Reports on successful therapeutic interventions have generally been small and inconsistent. [clinicaltrials.gov]
There is no one treatment that works for everyone, and the condition is chronic. However, there are several treatment options that may help control the condition and improve the skin’s appearance. People with suspected NL should see a doctor. [medicalnewstoday.com]
Infliximab: a promising new treatment option for ulcerated necrobiosis lipoidica. Dermatology. 2003. 206(2):180-1. [QxMD MEDLINE Link]. Kukreja T, Petersen J. Thalidomide for the treatment of refractory necrobiosis lipoidica. [emedicine.medscape.com]
The following treatments have helped some patients: The most common treatments are strong steroid creams or ointments, or calcineurin inhibitors (tacrolimus 0.03% or 0.1% ointment). [skinhealthinfo.org.uk]
Prognosis
As the management of necrobiosis lipoidica is not very reassuring, its prognosis is also not very satisfactory. [ncbi.nlm.nih.gov]
Outlook (Prognosis) NLD is a long-term disease. Lesions do not heal well and can recur. Ulcers are difficult to treat. The appearance of the skin may take a long time to become normal, even after treatment. [ucsfhealth.org]
Sections Continuing Education Activity Introduction Etiology Epidemiology Pathophysiology Histopathology History and Physical Evaluation Treatment / Management Differential Diagnosis Prognosis Complications Deterrence and Patient Education Enhancing Healthcare [pubmed.ncbi.nlm.nih.gov]
Prognosis Treatment for necrobiosis lipoidica is not very satisfactory. The disease is typically chronic with variable progression and scarring. [emedicine.medscape.com]
Etiology
Etiology Despite extensive studies, the etiology of necrobiosis lipoidica is still uncertain. [emedicine.medscape.com]
Etiology The cause of necrobiosis lipoidica remains unknown. [ncbi.nlm.nih.gov]
Sections Continuing Education Activity Introduction Etiology Epidemiology Pathophysiology Histopathology History and Physical Evaluation Treatment / Management Differential Diagnosis Prognosis Complications Deterrence and Patient Education Enhancing Healthcare [pubmed.ncbi.nlm.nih.gov]
Epidemiology
Sections Continuing Education Activity Introduction Etiology Epidemiology Pathophysiology Histopathology History and Physical Evaluation Treatment / Management Differential Diagnosis Prognosis Complications Deterrence and Patient Education Enhancing Healthcare [pubmed.ncbi.nlm.nih.gov]
The prevalence of other comorbidities were also higher than in the Jockenhöfer study and included the following [11] : Obesity - 51.6% Hypertension - 45.2% Dyslipidemia - 43.6% Thyroid disease - 24.5% Epidemiology The average age of onset for necrobiosis [emedicine.medscape.com]
Additionally, a correlation was noted between celiac disease and necrobiosis lipoidica, and it was observed that a larger percentage of patients having necrobiosis lipoidica had raised levels of thyroid antibodies.[7] Epidemiology Necrobiosis lipoidica [ncbi.nlm.nih.gov]
Pathophysiology
Pathophysiology[edit] Although the exact cause of this condition is not known, it is an inflammatory disorder characterised by collagen degeneration, combined with a granulomatous response. [en.wikipedia.org]
Sections Continuing Education Activity Introduction Etiology Epidemiology Pathophysiology Histopathology History and Physical Evaluation Treatment / Management Differential Diagnosis Prognosis Complications Deterrence and Patient Education Enhancing Healthcare [pubmed.ncbi.nlm.nih.gov]
This activity reviews the pathophysiology of necrobiosis lipoidica and highlights the role of the interprofessional team in ensuring the best patient outcomes. Objectives: Describe the pathophysiology of necrobiosis lipoidica. [ncbi.nlm.nih.gov]
Prevention
Avoid injury to the area to prevent the lesions from turning into ulcers. If you develop ulcers, follow steps on how to take care of the ulcers. If you smoke, you will be advised to quit. Smoking can slow healing of the lesions. [ucsfhealth.org]
The monoclonal antibodies adalimumab and infliximab bind directly to soluble TNF-alpha to prevent its action. Etanercept is a fusion protein made up of the Fc portion of human IgG1 and TNF receptors that also inhibit TNF function. [ncbi.nlm.nih.gov]
[…] and dipyridamole has been tried owing to a belief that necrobiosis lipoidica results from platelet-mediated vascular occlusion or immune mechanisms that alter platelet survival. [7] These drugs are thought to prolong platelet survival time and, hence, prevent [emedicine.medscape.com]