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Necrobiosis lipoidica
Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports
Necrobiosis lipoidica (NL) is a chronic cutaneous granulomatous condition with degenerative connective tissue changes. It is only seen in 1 in 300 diabetics but may be unassociated with glucose intolerance. NL may rarely be complicated by ulceration and squamous cell carcinoma.
Management Strategy
Smoking cessation and avoiding trauma to the affected shins are key factors to avoid transformation from an unsightly plaque into a painful, recalcitrant ulcer. The progression of new lesions may be halted by intralesional or occluded potent topical corticosteroids applied to the margins of the lesions. Topical tacrolimus can also be effective. Once atrophy has developed, there is little that will reverse this, although topical retinoids may be tried. Telangiectasia is often marked and has been treated with pulsed dye laser . Extensive lesions may justify trials of nicotinamide or prednisolone. Antiplatelet therapy in the form of aspirin or dipyridamole has its enthusiasts, though responses are inconclusive. Phototherapies such as topical psoralen and ultraviolet (UV) A (PUVA) and UVA-1 , as well as photodynamic therapy, have received recent interest and may arrest progression and improve the appearance. A variety of systemic antiinflammatory and immunosuppressive agents have been tried with some success, including mycophenolate mofetil, fumaric acid esters, ciclosporin, antimalarials, thalidomide, and pentoxifylline. Biologics, especially anti-TNFs (adalimumab, infliximab, and etanercept), and more recently ustekinumab , have also been proposed. Platelet-rich plasma has some evidence of efficacy.
The chronically ulcerated lesion is a challenge; antibiotics deal with secondary infection, appropriate dressings may be required, and growth factors such as becaplermin and granulocyte–macrophage colony-stimulating factor (GM-CSF) may accelerate healing. Because diabetics may have coexisting large vessel atherosclerosis that may contribute to ulceration, non-invasive arterial studies or angiography needs to be considered if clinically indicated.
Excision and grafting including punch grafting may transform the patient’s quality of life and improve cosmesis. Work with the diabetologist to optimize diabetic control.
Specific Investigations
Squamous cell carcinoma arising in an area of long-standing necrobiosis lipoidica
Lim C, Tschuchnigg M, Lim J. J Cutan Pathol 2006; 33: 581–3.
Case report of a squamous cell carcinoma arising de novo in an area of NL.
Unilateral necrobiosis lipoidica of the ischemic limb – a case report
Naschitz JE, Fields M, Isseroff H, et al. Angiology 2003; 54: 239–42.
A possible ischemic pathogenesis of NL emerges from a case of unilateral large vessel arteriosclerotic ischemia with ipsilateral NL. The authors report a severely ulcerated area of NL that only started to heal after the opening of severe femoropopliteal atheroma.
Updated results of 100 patients on clinical features and therapeutic options in necrobiosis lipoidica in a retrospective multicenter study
Erfurt-Berge C, Dissemond J, Schwede K, et al. Eur J Dermatol 2015; 25: 595–601.
This multicenter retrospective study included 100 patients with NL lesions on the lower leg. Thyroid disorders were found in 15% of all cases.
Necrobiosis lipoidica diabeticorum: association with background retinopathy, smoking, and proteinuria. A case controlled study
Kelly WF, Nicholas J, Adams J, et al. Diabet Med 1993; 10: 725–8.
Fifteen diabetics with NL were each matched with five control subjects with diabetes mellitus. Background retinopathy, proteinuria, and smoking were all more common with NL. No differences were noted between those with NL and controls in the prevalence of vascular disease and neuropathy. Glycosylated hemoglobin concentrations were higher in patients with NL.
Stop smoking and control diabetes mellitus.
Granuloma annulare and necrobiosis lipoidica treated by jet injector
Sparrow G, Abell E. Br J Dermatol 1975; 93: 85–9.
Three of five cases of NL underwent complete resolution, and one had partial improvement with 5 mg/mL triamcinolone injection to the edges of lesions. No serious complications of this type of treatment were observed.
Treatment of psoriasis and other dermatoses with a single application of a corticosteroid left under a hydrocolloid occlusive dressing for a week
Juhlin L. Acta Dermatol Venereol 1989; 69: 355–7.
A 0.1% betamethasone alcoholic lotion under a hydrocolloid dressing was an effective, well-tolerated treatment, and three applications only were required.
Necrobiosis lipoidica: treatment with systemic corticosteroids
Petzelbauer P, Wolff K, Tappeiner G. Br J Dermatol 1992; 126: 542–5.
Oral methylprednisolone was given to six patients with non-ulcerating NL for 5 weeks; in all there was cessation of disease activity during the 7-month follow-up period. Initial dosage was 1 mg/kg daily for 1 week, then 40 mg daily for 4 weeks, followed by tapering and termination in 2 weeks. All patients, including the diabetics, tolerated the treatment well. There was no improvement in atrophy. Benefit was maintained at the end of the 7-month follow-up period.
Careful monitoring of blood glucose is mandatory in all diabetic patients with NL treated with systemic corticosteroids.
Pentoxifylline: an effective therapy for necrobiosis lipoidica
Wee E, Kelly R. Australas J Dermatol 2015 Nov 12 [Epub ahead of print].
Three cases of biopsy-proven NL were treated with pentoxifylline 400 mg three times daily. Patients 1 and 2 had early-stage NL, presenting with indurated, red-brown plaques. Pentoxifylline reversed the inflammation and induration completely after 7 and 12 months, respectively. No recurrence in activity was seen in patient 1 after 12 months. In patient 3, pentoxifylline was effective in reversing ulceration completely after 10 months of treatment. Some areas of atrophy remained in all patients. None of the patients reported significant side effects.
Healing of necrobiotic ulcers with antiplatelet therapy. Correlation with plasma thromboxane levels
Heng MC, Song MK, Heng MK. Int J Dermatol 1989; 28: 195–7.
NL in diabetics has been considered a cutaneous manifestation of diabetic microangiopathy. Seven diabetic patients with necrobiotic ulcers of recent onset that healed after administration of 80 mg/day of acetylsalicylic acid and 75 mg three times daily of dipyridamole had elevated thromboxane levels. Healing was associated with depression of the elevated thromboxane levels in all seven patients.
Treatment of necrobiosis lipoidica with low-dose acetylsalicylic acid. A randomized double-blind trial
Beck HI, Bjerring P, Rasmussen I, et al. Acta Dermatol Venereol 1985; 65: 230–4.
No response was seen with the use of 40 mg acetylsalicylic acid daily for 24 weeks, despite documented platelet aggregation inhibition.
High dose nicotinamide in the treatment of necrobiosis lipoidica
Handfield-Jones S, Jones S, Peachey R. Br J Dermatol 1988; 118: 693–6.
An open study of high-dose nicotinamide (1.5 g/day) in the treatment of 15 patients with NL. Of 13 patients who remained on treatment for more than 1 month, eight improved. A reduction in pain, soreness, and erythema, and the healing of ulcers if present, was noted. There were no significant side effects, particularly with respect to diabetic control. Lesions tended to relapse if treatment was stopped.
Clofazimine – therapeutic alternative in necrobiosis lipoidica and granuloma annulare
Mensing H. Int J Dermatol 1989; 28: 195–7.
Ten patients with NL were treated with clofazimine 200 mg orally daily. Six of 10 patients responded: three of the responders achieved complete remission of the dermatosis. All the patients treated had reddening of the skin, but this was reversible after the end of therapy, as were the other side effects (i.e., diarrhea and dryness of the skin).
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