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NECROBIOSIS LIPOIDICA DIABETICORUM (NLD) (Clinical Photo 1 )

The incidence is 0.3% in diabetics, and it is rare in non-diabetics. The condition is most common between the second and fifth decades of life, but it may be seen at any age. 80% of patients with NLD are women. NLD occurs almost exclusively in whites.

between 60 and 65% with NLD will have overt diabetes at the time of the diagnosis. Of the remainder of patients, about 50% will show abnormalities when challenged by routine or cortisone glucose tolerance tests. Another 25% of patients will have a strong family history of diabetes. This leaves only some 10% of the total number of patients who lack a diabetic association.

The primary lesion of NLD is a well-defined, small, firm, dusky-red papule topped with a fine scale. By slow enlargement or coalescence, these lesions form indurated plaques that are round or oblong when small and have an irregular geographic configuration when larger. The border, which sometimes is slightly elevated, and the adjoining skin are reddish-blue, whereas the center is yellow, indicating lipid accumulation. The size of the lesion may vary from a few millimeters to several centimeters. The inflammatory process subsides, and the condition assumes its best recognized, chronic state, that is, the sharply demarcated sclerotic plaque reminiscent of glazed porcelain. The glossy atrophic area softens and becomes entirely brown. Through its surfaces numerous telangiectases and underlying larger blood vessels can be seen.

The scale may remain fine or, particularly if ulceration is imminent, become more prominent and collodion-like. ulceration occurs in approximately one third of patients regardless of whether they are diabetic. It is more common in larger lesions and may follow trauma.

Lesions of NLD are most frequent on the lower portions of the legs, the pretibial and medial malleolar areas being the favored sites. Lesions occasionally appear on the thighs, popliteal regions, and feet. In 15% of cases other sites are involved in addition to the legs. These sites include the abdomen, upper extremities(especially the hands and forearms), and scalp, where NLD can cause atrophy and alopecia, and the face, including the eyelids and nose. In rare cases the condition has been noted on the heels or penis. necrobiosis lipoidica diabeticorum also has developed in scars and at sites of scleroderma and BCG vaccinations. Even when the lesions appear elsewhere on the body, the legs generally are also involved.

except when they are ulcerated, the papules and plaques are generally asymptomatic. The occasional patient will have pruritus, burning, or tenderness. Pain, however, is a frequent companion of ulceration. Some patients report partial or complete anesthesia of the affected sites, suggesting local nerve dysfunction.

As many as one in five lesions will resolve spontaneously, the time required for improvement varies from 3 to 4 years.

Treatment: The physician should stress the probability of localization of lesions to the low part of the legs, the absence of contagion, lack of malignant degeneration, and the possibility that some areas will heal spontaneously. In patients with overt diabetes, adequate follow-up tests for urine and blood glucose levels are important.

The lower parts of the legs should be protected from trauma. Patients should be advised to avoid potentially traumatizing situations such as certain sports and they should wear knee-length stockings or shin pads for protection.

In general, although many treatments have been touted, they have little in the way of proven efficacy, and should probably be reserved for symptomatic relief.

Topical steroids under occlusion has been used but has not been subjected to double-blind studies. Dermajet delivery of triamcinolone acetonide (TMC) has shown improvement in an open study and intralesional injection of 0.1 cc of TMC, 2.5 mg/ml, perilesionally at 1 cm intervals has been suggested for active plaques. Clofazimine 200 mg/day cleared 6/10 patients, of 13 patients who remained on treatment for more than one month, eight improved

Clinical Associations:

Necrobiosis lipoidica has been seen in Ehler's-Danlos Syndrome, type VIII (OMIM ) as well as Ataxia-Telangiectasia (Thibaut )

Thibaut, S.; Sass, U.; Khoury, A. and Simonart, J.-M.:

Ataxia-telangiectasia and necrobiosis lipoidica: an explainable association. Europ. J. Derm. 4: 509-513, 1994.