Necrobiosis Lipoidica Treatment & Management

Treatment for necrobiosis lipoidica is not very effective, partially because the exact etiology remains unknown. Because localized trauma can cause necrobiosis lipoidica to ulcerate, protection of the legs with elastic support stockings and leg rest may be helpful.

Topical and intralesional corticosteroids can lessen the inflammation of early active lesions and the active borders of enlarging lesions but have little beneficial effect on so-called “burned out” atrophic lesions. In fact, with atrophic lesions, corticosteroid use may cause further atrophy.

In 2005, Clayton and Harrison reported a case of ulcerated necrobiosis lipoidica that was treated successfully with 0.1% topical tacrolimus ointment applied twice daily for 1 month.^[7] Tacrolimus is a calcineurin inhibitor that has been shown to have a mechanism of action similar to that of cyclosporine in that it prevents T-cell activation. Cyclosporine at doses of 2.5 mg/kg/day has also been used with success in treating ulcerated necrobiosis lipoidica.^[8]

Spencei and Nahass described a case of ulcerated necrobiosis lipoidica that was treated successfully with topically applied bovine collagen.^[9] Collagen is believed to improve granulation tissue by supporting fibroblast activity and by promoting wound debridement by increasing the number of macrophages and neutrophils at the wound site.

De Rie et al reported successful treatment of necrobiosis lipoidica with topical psoralen plus ultraviolet-A (UVA) light therapy.^[10] Thirty patients were treated with twice-weekly courses of topical psoralen plus UVA light therapy. Five patients had complete clearing of their ulceration and erythema, and 11 patients showed significant improvement in their disease. UVA-1 and photodynamic therapy have been reported to be as successful.^{[11, 12]}

Ticlopidine,^[13] nicotinamide,^[14] clofazimine,^{[15, 16]} and perilesional heparin injections^[17] have been used in uncontrolled studies and appeared to benefit some patients with necrobiosis lipoidica.

According to W.R. Heymann, tretinoin has been used to diminish the atrophy associated with necrobiosis lipoidica (personal communication).^[18]

Durupt et al reported successful treatment of necrobiosis lipoidica with chloroquine and hydroxychloroquine. Improvement was seen within 3- 6 months of treatment.^[19]

Surgical and laser therapy

Excision and grafting have been successful, but recurrence may take place secondary to the underlying vascular damage. Poor healing of the graft site is not uncommon.^[20]

Laser care is also described. Moreno-Arias and Camps-Fresneda treated necrobiosis lipoidica with a pulse dye laser (Candela SPTL; Irvine, Mass).^[21] They reported overall cosmetic improvement after 3 treatment sessions with respect to erythema and telangiectasis. Stabilization of the lesions was also achieved with the laser treatments.

Antiplatelet aggregation therapy with aspirin 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.^[22] These drugs are thought to prolong platelet survival time and, hence, prevent further worsening of necrobiosis lipoidica. The results of double-blind studies with aspirin and dipyridamole have varied but overall have shown some beneficial effects from the therapy.

Littler and Tschen reported a case of necrobiosis lipoidica that was treated successfully with pentoxifylline, a drug used in the treatment of intermittent claudication.^[23] Pentoxifylline not only inhibits platelet aggregation but is also believed to decrease blood viscosity by increasing fibrinolysis and red blood cell deformity.

As previously mentioned, TNF-alpha may have a critical role in conditions such as disseminated granuloma annulare and necrobiosis lipoidica. It occurs in high concentrations in the sera and skin in patients with these conditions.

Boyd reported that the thiazolidinedione drug class has been used with some success in necrobiosis lipoidica. These drugs function as potent agonists for the peroxisome proliferator–activated receptor-gamma (PPARgamma). These receptors have been found in adipose tissue and function as important mediators in lipid storage and adipocyte differentiation.^[24] PPARgamma activation diminishes TNF-alpha production and inhibits the action of proinflammatory cytokines, which may help healing in necrobiosis lipoidica.^{[25, 26]}

In several case reports, the TNF-alpha inhibitors etanercept, adalimumab, and infliximab were shown to improve chronic granulomatous skin disorders.^{[27, 28]} Infliximab is a chimeric monoclonal antibody that acts to inhibit TNF-alpha. Kolde et al reported infliximab as a successful treatment option for ulcerated necrobiosis lipoidica.^[29] Successful treatment of necrobiosis lipoidica with thalidomide, another drug with anti-TNF effects, has also been reported.^[30]