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Necrobiosis lipoidica is an inflammatory skin disorder characterised by irregularly shaped, callous lesions with reddish-brown pigmentation and central atrophy. It was originally described in conjunction with diabetes - in 1929, by Oppenheim who coined the name dermatitis atrophicans lipoidica diabetica and, in 1932, by Urbach who called it necrobiosis lipoidica diabetica. Today the term is applied to all cases, whether occurring in diabetics or not. Necrobiosis lipoidica has also been reported in association with inflammatory bowel disease, granuloma annulare and sarcoid.
The pathology is collagen degeneration with granulomatous response, associated with thickened blood vessels and fat deposition. The underlying cause is unknown: aetiology has suggested an antibody-mediated vasculitis with secondary collagen degeneration or diabetic microangiopathy.
It is a rare skin condition. Whilst there is a high prevalence of diabetes mellitus in patients with necrobiosis lipoidica (50% occurs in diabetics), the reported prevalence of necrobiosis lipoidica in diabetic patients is 0.3%. It most commonly presents in the 30s but can present at any age, including infancy. It tends to appear earlier in diabetics than in others: in one study, approximately 2% of young diabetics (aged up to 22 years) had a necrobiosis lipoidica lesion compared with none of the control subjects. It is 3 times as common in women as in men. Nondiabetic familial clustering of necrobiosis lipoidica does occur but extremely rarely.
Another study found that necrobiosis lipoidica preceded the diagnosis of diabetes in 15% of patients. In 25%, the lesions were present with the onset of diabetes.
Smoking is more prevalent in diabetic patients with necrobiosis lipoidica than without, as are diabetic complications (retinopathy and nephropathy) although the presence of necrobiosis lipoidica does not correlate with diabetic control.
- Shiny patches slowly enlarge over months or years. They are initially a reddish brown and 1-3 mm in diameter but progress to yellow and become depressed and atrophic plaques.
- The most common site is the pretibial area but they can occur on the face, scalp, trunk and upper arms where they are less likely to be correctly diagnosed.
- In about 75% of cases there is no pain (due to associated neuropathy) but they may be very painful.
- Trauma produces ulceration.
- They may display the Koebner phenomenon in which lesions occur in areas of trauma. (This phenomenon is more typically associated with psoriasis and lichen planus.)
Usually the appearance is fairly typical but variations can be difficult to diagnose. Consider as a cause of atypical leg ulcers in diabetic patients.
- Superficial annular lesions may look like granuloma annulare.
- Yellow, fatty lesions may resemble xanthoma.
- Sarcoidosis can appear very similar, even on histology.
- Rheumatoid nodules are similar histologically but tend to be raised rather than atrophic. Ulcerated necrobiotic areas have been described in rheumatoid arthritis.
- Varicose eczema produces a scaly rash and is usually near the malleoli.
If the patient is not known to be diabetic this must be checked. Biopsy of the lesion may be helpful but be aware of poor wound healing.
There is much surrounding redness but the small yellow ulcer is the pertinent bit.
Management is impaired by lack of understanding of the aetiology of the condition. No treatment to date is completely effective and, whilst numerous treatments have been tried, none has proven effectiveness based on controlled trials.
- Trauma should be avoided.
- Traditionally, the mainstream view has been that improving diabetic control is not beneficial to necrobiosis lipoidica lesions but this has been challenged and tight diabetic control may reduce the incidence of this rare disease and possibly aid healing. Since smoking is more common in affected adults, it would seem wise to counsel against it. Use of glitazones has benefited necrobiosis lipoidica lesions in some cases.
- Topical or intralesional steroids may reduce inflammation but it does not benefit burned-out lesions and may aggravate atrophy. Courses of systemic corticosteroids have also been used but are controversial, not least since they may upset glycaemic control.
- Antiplatelet treatment seems logical but controlled trials have had different results. Aspirin and dipyridamole have been used. Pentoxifylline decreases blood viscosity and increases fibrinolysis and erythrocyte deformity and it may be helpful. Ticlopidine and perilesional injections of heparin have been used in uncontrolled trials.
- Phototherapy - ultraviolet A1 (UVA1) - has been as an adjunct to other forms of treatment.
- Immunomodulating drugs have also been used to treat necrobiosis lipoidica: ciclosporin, topical tacrolimus and antitumour necrosis factor-α (anti-TNF-α) therapies with varying levels of success. Interestingly, a case study describes spontaneous healing of necrobiosis lipoidica following pancreas and kidney transplantation and the immunosuppressive regime is thought to have played a significant role in this improvement.
- One study reported successful use of bovine collagen which was thought to support fibroblast activity and encourage debridement by bringing macrophages and neutrophils to the wound site.
- Excision and grafting are occasionally used but poor healing and recurrence are common.
- Laser treatment has been used to stabilise lesions and reduce erythema and telangiectasis.
The lesions do not heal well and are usually considered a chronic, relapsing condition. They are known to remit spontaneously and even resolve. Spontaneous remission with residual scars and atrophy was observed in 17% of patients but flare-ups are to be expected. Occasionally squamous cell carcinoma can arise in areas of longstanding necrobiosis lipoidica.
Further reading & references
- Necrobiosis lipoidica, Diabetes UK
- Cohen O, Yaniv R, Karasik A, et al; Necrobiosis lipoidica and diabetic control revisited. Med Hypotheses. 1996 Apr;46(4):348-50.
- Barnes CJ; Necrobiosis lipoidica, eMedicine, July 2009.
- Pavlovic MD, Milenkovic T, Dinic M, et al; The prevalence of cutaneous manifestations in young patients with type 1 diabetes. Diabetes Care. 2007 Aug;30(8):1964-7. Epub 2007 May 22.
- Ho KK, O'Loughlin S, Powell FC; Familial non-diabetic necrobiosis lipoidica. Australas J Dermatol. 1992;33(1):31-4.
- Kelly WF, Nicholas J, Adams J, et al; Necrobiosis lipoidica diabeticorum: association with background retinopathy, smoking, and proteinuria. A case controlled study. Diabet Med. 1993 Oct;10(8):725-8.
- Gottrup F, Karlsmark T; Leg ulcers: uncommon presentations. Clin Dermatol. 2005 Nov-Dec;23(6):601-11.
- Boyd AS; Thiazolidinediones in dermatology. Int J Dermatol. 2007 Jun;46(6):557-63.
- Rallis E, Korfitis C, Gregoriou S, et al; Assigning new roles to topical tacrolimus. Expert Opin Investig Drugs. 2007 Aug;16(8):1267-76.
- Gullo D, Latina A, Tomaselli L, et al; Healing of chronic necrobiosis lipoidica lesions in a type 1 diabetic patient after pancreas-kidney transplantation: a case report. J Endocrinol Invest. 2007 Mar;30(3):259-62.
- Souza AD, El-Azhary RA, Gibson LE; Does pancreas transplant in diabetic patients affect the evolution of necrobiosis Int J Dermatol. 2009 Sep;48(9):964-70.
- Boulton AJ, Cutfield RG, Abouganem D, et al; Necrobiosis lipoidica diabeticorum: a clinicopathologic study. J Am Acad Dermatol. 1988 Mar;18(3):530-7.
- Lim C, Tschuchnigg M, Lim J; Squamous cell carcinoma arising in an area of long-standing necrobiosis lipoidica. J Cutan Pathol. 2006 Aug;33(8):581-3.
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|Original Author: Dr Chloe Borton||Current Version: Dr Laurence Knott|
|Last Checked: 20/04/2011||Document ID: 3001 Version: 23||© EMIS|