Necrobiosis Lipoidica With or Without Diabetes
Medically reviewed by Kelly Wood, MDMedically reviewed by Kelly Wood, MDNecrobiosis lipoidica is a rare skin ailment that often begins on the lower legs and shins. Though many of those affected have type 1 or type 2 diabetes, the rash can also occur in people without diabetes. It is also linked with many other systemic (whole-body) diseases.
Reproduced with permission from © DermNet dermnetnz.org 2023.
Necrobiosis Lipoidica Diabeticorum RashNecrobiosis lipoidica diabeticorum rash occurs in people with type 1 or type 2 diabetes. Though this rash is linked to diabetes, it is unrelated to the control of blood glucose.Reason It HappensThe reason that necrobiosis lipoidica diabeticorum rash happens is not well understood. Theories center on inflammation and other changes that affect blood vessels associated with autoimmune diseases (conditions in which your body attacks its own cells) such as diabetes or antibody-mediated vasculitis (inflamed blood vessels). These factors can deteriorate collagen proteins in your skin.Your risk of developing necrobiosis lipoidica increases if you have any of the following characteristics:Female: Research indicates a high predominance of females affected by the disease, with one study reporting up to 77% of people affected are female.Type 1 or type 2 diabetes: Though people with type 1 diabetes are more likely to develop the rash, it can also affect people who have type 2 diabetes. Fewer than 1% of people with diabetes will develop necrobiosis lipoidica diabeticorum rash. From 11% to 65% of people with necrobiosis lipoidica also have diabetes.Age of 30 to 40 years: Though the disease can occur at any time, it most commonly occurs during these decades. Research indicates that the age of onset is lower for patients with type 1 diabetes than for those with type 2 diabetes.
Learn More: The Effects of Diabetes on Skin
Identifying SymptomsSymptoms of necrobiosis lipoidica diabeticorum rash can vary during the disease and among different people. They can include:One to three well-defined painless papules (firm, smooth bumps) or nodules (lumps beneath the skin) at onset, typically occurring on the lower legs or shinsWith progression, the lesions flatten and develop well-defined red borders, gradually merging into oval-shaped plaques with a reddish-brown core that changes to yellowish-brown discolorationPain and itchiness with lesion progressionAtrophy (wasting away), waxiness, and open sores in the central region of the plaquesRarely, the appearance of lesions elsewhere, such as the upper extremities, hands, torso, groin, face, or scalpPeriods of disease activity, or flares, and inactivity
Learn More: Diabetic Dermopathy: Pictures, Symptoms, Treatment
Necrobiosis Lipoidica Treatment and EffectivenessBecause necrobiosis lipoidica is rare and its causes are not fully understood, no conclusive treatment regimens exist for this condition. Treatment plans are individualized and can involve several modalities before results are achieved. The condition is a long-term disease that can heal and recur.Treatment is individualized and may require modifications as the disease progresses. Common treatments for necrobiosis lipoidica include:CorticosteroidsCorticosteroids administered as topical, intralesional, and sometimes systemic, are considered a first-line treatment for the rash. In one multicenter study, researchers reported that topical corticosteroids had a positive effect in 40% of cases treated.However, topical corticosteroids can delay wound healing and aggravate some skin infections, especially atrophied lesions. Prolonged use can exacerbate hypertension (high blood pressure) and hyperglycemia (high blood sugar), which can complicate disease control in people with diabetes. Therefore, the use of systemic corticosteroids in people with diabetes and necrobiosis lipoidica is controversial.There is a wide range of second-line treatments for necrobiosis lipoidica, but there is no definitive evidence that supports one therapy over others. In an analysis of 16 treatment regimens in 49 publications, researchers concluded that there is no clear recommendation for a specific second-line therapy when corticosteroids fail. Some notable treatment options include:Calcineurin InhibitorsResearch indicates that calcineurin inhibitors, specifically tacrolimus ointment, are more effective than topical corticosteroids. Results of one study showed that tacrolimus was effective in 61.5% of cases, yielding better results than corticosteroids without the risk of skin atrophy inherent in corticosteroids. Tacrolimus is also effective in treating ulcers that occur with the condition.Fumaric Acid EstersResults from a prospective, uncontrolled study of 18 people with necrobiosis lipoidica showed that the administration of fumaric acid esters over six months yielded significant clinical improvement. Though the results were notable, moderate side effects including gastrointestinal upset and flushing occurred.Photodynamic TherapyTreatment with photodynamic therapy has been successful as a second-line treatment for necrobiosis lipoidica in some cases. Researchers have reported complete responses in up to 66% of people treated with photodynamic therapy, and partial responses in 39% to 90% of cases treated.Other DrugsOther drugs reported to have varying worthwhile impact on the disease include the following:Antimalarials (chloroquine, hydroxychloroquine)CyclosporineDoxycyclinePentoxifyllineTumor necrosis factor (TNF) inhibitors including Humira (adalimumab), Remicade (infliximab), and Enbrel (etanercept)Newer TreatmentsThough the evidence is limited, newer treatments have proved effective in the treatment of necrobiosis lipoidica in single cases and warrant further investigation in larger groups of people affected by the condition. These treatments include:Biologics including Stelara (ustekinumab) and Cosentyx (secukinumab)Janus kinase (JAK) inhibitors including Xeljanz (tofacitinib), Jakafi (ruxolitinib), Olumiant (baricitinib), and CIBINQO (abrocitinib),Aryl hydrocarbon receptor (AhR) agonists including Vtama (tapinarof)Treatment of lesions with platelet-rich plasma in gel form applied to the lesionsHyperbaric oxygen therapySurgical resection of a necrobiosis lipoidica lesion followed by a skin graft
Risks/Complications of Necrobiois LipoidicaThe main risks and complications reported in necrobiosis lipoidica involve:Development of malignancy, typically in the form of squamous cell carcinoma, in the lesionSecondary infection or painful ulceration in the wound bed, often a result of trauma to the siteUnsightly scarring at the site, which can create a cosmetic disability
Necrobiosis Lipoidica Without DiabetesThe onset of necrobiosis lipoidica is not limited to people with diabetes. This skin condition has been linked with other diseases, though the absence of diabetes does not seem to have a significant impact on many aspects of the disease.Reasons It HappensAmong people who have necrobiosis lipoidica, research indicates that the lesions in people with and without diabetes share many clinical features. This association suggests that factors other than elevated blood glucose may contribute to the disease's onset.Though necrobiosis lipoidica has been linked with diabetes, it is also associated with systemic conditions that include the following, though the reason for the link is unclear:ObesityHypertension (high blood pressure)Hyperlipidemia (elevated blood lipids, such as cholesterol and triglyceride)Thyroid diseaseIn a study comparing the experiences of people with diabetes and those without it, necrobiosis lipoidica occurred at a younger age and among more females in people with diabetes versus those without diabetes.Identifying SymptomsResearch indicates that symptoms of necrobiosis lipoidica do not seem to have notable variation between people with or without diabetes. However, there is some evidence that ulceration, a risk that occurs in up to 35% of all cases of necrobiosis lipoidica, happens more often in affected people with diabetes.Healing TimeThough treatments remain the same regardless of whether diabetes is present or not, people without diabetes do not have the burden of considering the impact on disease control. Because hyperglycemia is a side effect of systemic corticosteroids, people without diabetes may be able to continue this type of first-line treatment without controversy.Necrobiosis lipoidica is a chronic, incurable disease in which lesions do not heal well. Plaque patches can take years to heal. Recurrence is possible. Even with prompt and appropriate treatment, scarring and discoloration are likely. When possible, the appearance of affected skin can take a long time to appear normal.
Learn More: Getting a Foot Ulcer to Heal
How to Manage Chronic Necrobiosis LipoidicaNecrobiosis lipoidica is a long-term disease. Generally, treatment goals are to reduce symptoms and decrease the risk of ulceration. The risk of ulceration is more likely with late diagnosis and delayed treatment.Lifestyle adjustments can complement medical treatments to manage chronic necrobiosis lipoidica. Common advised lifestyle modifications include the cessation of smoking and the avoidance of trauma in the affected area.Protecting non-ulcerated lesions on the lower legs can be achieved by utilizing support stockings to prevent trauma and the occurrence of Koebner's phenomenon (the appearance of new skin lesions on previously healthy skin after trauma).Adherence to appropriate wound care guidelines is crucial in cases in which ulcerations are evident. Understanding symptoms and ways to reduce the risk of infection is vital. If you have been diagnosed with necrobiosis lioidica, notify your healthcare provider about signs of infection, a lump, an ulcer, or a persistent scab as soon as possible.
Learn More: Diabetic Ulcer: Improving Healing Success for Feet and Legs
SummaryNecrobiosis lipoidica is a chronic skin disease often linked with diabetes. It can also occur in healthy people or those with other systemic health problems such as thyroid disease.Necrobiosis lipoidica causes lesions that form patches on your lower legs or shins. Concerns include ulceration, scarring, and sometimes, cancer. Treatment is individualized because there is little knowledge of how the disease occurs. Though researchers have reported good outcomes with many types of treatments, few large studies exist to confirm their results.If you have been diagnosed with diabetes, contact your healthcare provider if you notice lesions on your lower shins or other signs of this disease. Early diagnosis and treatment can improve outcomes and reduce your risk of an ulcer, which can be a painful and severe complication. Read the original article on Verywell Health.
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