Necrobiosis lipoidica diabeticorum


Necrobiosis lipoidica diabeticorum

Description, Causes and Risk Factors:

Abbreviation: NLD.

A condition, in many cases associated with diabetes, in which one or more yellow, atrophic, shiny lesions develop on the legs (typically pretibial); characterized histologically by indistinct areas of necrosis in the cutis.

Necrobiosis lipoidica diabeticorum is a rash that occurs on the lower legs. It is more common in women, and there are usually several spots. They are slightly raised shiny red-brown patches. The centers are often yellowish and may develop open sores that are slow to heal. Often a biopsy is needed to diagnose NLD.

NLD usually occurs more often in people with diabetes, in people with a family history of diabetes or a tendency to get diabetes. Still, the exact cause of NLD in not known. A similar condition that is often confused with NLD is granuloma annulare. Similar to the association of NLD and diabetes, it appears that a high percentage of persons with disseminated granuloma annulare have diabetes mellitus. The individual spots typically consist of a circular array of reddish to brown and slightly translucent bumps.

An additional etiologic theory focuses on the abnormal collagen in necrobiosis lipoidica. It is well established that abnormal and defective collagen fibrils have been responsible for diabetic end-organ damage and accelerated aging. Lysyl oxidase levels have been found in some diabetic persons to be elevated and are responsible for increased collagen cross-linking. Increased collagen cross-linking could explain basement membrane thickening in necrobiosis lipoidica.

Other theories link trauma and inflammatory and metabolic changes as a possible etiology. It also has been found that there may be impaired neutrophil migration leading to an increased number of macrophages, possibly explaining the granuloma formation in necrobiosis lipoidica. The pathogenesis of necrobiosis lipoidica has not been demonstrated to be linked to genetic factors.

Tumor necrosis factor (TNF)-alpha has a potentially critical role in conditions such as disseminated granuloma annulare and necrobiosis lipoidica. It is found in high concentrations in the sera and skin in patients with these conditions.

The average age of onset for necrobiosis lipoidica is 30 years, but it can occur at any age. The age of onset ranges from infancy to the eighth decade. The disease tends to develop at an earlier age in patients with diabetes. It also shows a sex predilection, being 3 times more common in women than in men.

Necrobiosis lipoidica has been described in about 0.3% of diabetic patients. In one study, necrobiosis lipoidica was shown to precede the onset of diabetes mellitus in 15% of patients. In addition, 60% of patients had the diagnosis of diabetes mellitus prior to the onset of necrobiosis lipoidica, while 25% of patients had lesions that appeared with the onset of diabetes mellitus. The presence or progression of necrobiosis lipoidica does not correlate with how well diabetes is controlled.

Symptoms:

NLD appears as a rash most often on the lower legs, but some people might notice it on their face, torso, scalp or arms.

In the beginning, the rash might appear reddish-brown with fairly well-defined borders. Over time, the lesions might grow larger and turn shiny and red, sometimes developing a yellow center. Eventually, the lesion develops into a purplish depression in the skin.

Sometimes, NLD itches and hurts; but many people report no symptoms other than the rash.

Diagnosis:

NLD is diagnosed by a skin biopsy, demonstrating superficial and deep perivascular and interstitial mixed inflammatory cell infiltrate (including lymphocytes, plasma cells, mononucleated and multinucleated histiocytes, and eosinophils) in the dermis and subcutis, as well as necrotising vasculitis with adjacent necrobiosis and necrosis of adnexal structures. Areas of necrobiosis are often more extensive and less well defined than in granuloma annulare. Presence of lipid in necrobiotic areas may be demonstrated by Sudan stains. Cholesterol clefts, fibrin, and mucin may also be present in areas of necrobiosis. Depending on the severity of the necrobiosis, certain cell types may be more predominant. 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.

Direct immunofluorescence microscopy of necrobiosis lipoidica has demonstrated immunoglobulin M (IgM), IgA, C3, and fibrinogen in the blood vessels, which cause the vascular thickening. In nondiabetic patients with necrobiosis lipoidica, the vascular changes are not as prominent.

Treatment:

As long as the lesions of the rash do not break open, NLD does not usually require treatment. Your doctor might ask you to rest your legs occasionally and protect them with elastic support stockings. He or she might also recommend that you take a baby aspirin each day.

Treating NLD can be tricky. During a flare up, some people find relief using a topical cream that contains cortisone and covering the area with a sterile dressing. Others find that cortisone injections can help.

Some researchers have found that treating the area with ultraviolet light can control NLD during flare-ups. In some cases, doctors might prescribe steroids such as prednisone.

Trauma to the affected area might cause ulcers to form, and these do need treatment. If this occurs, see your doctor.

NOTE: The above information is for processing purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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