Necrobiosis Lipoidica Diabeticorum

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Overview

Lesions of necrobiosis lipoidica diabeticorum (NLD) are usually, but not always, associated with juvenile-onset diabetes. Tending to occur singly or in crops on the anterior lower legs, they can appear anywhere on the body, however. Lesions on the leg exhibit more of a tendency to ulcerate than lesions elsewhere.

First Steps

1. For nonulcerated, symptomatic lesions (asymptomatic lesions need not be treated), intralesional triamcinolone acetonide 3-5 mg/ml. Small amounts of this solution are injected intradermally into plaques via a 30-guage needle. Injections are repeated every 2-3 weeks.
2. Application of superpotent topical steroids with occlusion for several weeks can be effective.

Ancillary Steps

1. Administration of pentoxiphylline 400-800 mg three times plus ASA 81 mg daily.
2. For persistent erythema and telangiectasia, not resolving with the above therapy, pulse dye laser can be utilized for cosmetic improvement.

Subsequent Steps

1. Topical tacrolimus ointment 0.1% may be used for refractory lesions.
2. Topical or systemic PUVA may improve NLD, and can even be used in cases with ulceration, with reduction in pain after several treatments.
3. In severe refractory cases requiring systemic treatment, consider the following options:
   a. Cyclosporine 2.5-5 mg/kg per day for several months
   b. Mycophenolate mofetil 2-3 g/day
   c. Infliximab 5 mg/kg per treatment for a total of 3 treatments at 0, 2, and 6 weeks.
4. NLD has a tendency to ulcerate. These ulcerations may be infected by bacteria, so nonhealing lesions should be cultured, and appropriate topical and oral antibiotics should be considered if the methods outlined below are ineffective. At times the ulcerated lesions will be very painful. The techniques used to heal chronic ulcers can be applied to the ulcerated lesions of NLD, including these treatments:
   a. Application of semipermeable dressings
   b. Becaplermin applied once daily to the ulceration for 12 hours, or once weekly under occlusive, semipermeable dressing, as for venous insufficiency ulcers.
   c. Topical or intralesional granulocyte-macrophage colony-stimulating factor
   d. Dermagraft or other skin substitute equivalent applied at regular intervals. Multiple applications may be required. This is frequently very effective in reducing/eliminating the pain as well as stimulating ulcer healing.

Pitfalls

1. Lesions of NLD can be very resistant to the above management, and may not respond. Moreover, new lesions may continue to form.
2. There is no relationship between severity of NLD and diabetic status.

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