Journal of Postgraduate Medicine Medknow Publications and Staff Society of Seth GS Medical College and KEM Hospital, Mumbai, India ISSN: 0022-3859 EISSN: 0972-2823 Vol. 51, Num. 2, 2005, pp. 127-130

Journal of Postgraduate Medicine, Vol. 51, No. 2, April-June, 2005, pp. 127-130

Spot the diagnosis

Cutaneous lesions on the legs

Putta S. Manohar, Syed Akheel A., Parr J.H.

Department of Diabetes and Endocrinology, South Tyneside District Hospital, Harton Lane, South Shields, Tyne and Wear NE34 OPL

Correspondence Address:Department of Diabetes and Endocrinology, South Tyneside District Hospital, Harton Lane, South Shields, Tyne and Wear NE34 OPL,Email: a.a.syed@newcastle.ac.uk

Code Number: jp05050

A 34-year-old woman had several symmetrically located, well-circumscribed, non-ulcerating, waxy, red-brown plaques on her lower limbs [Figure - 1]. The first lesion appeared 13 years ago. She was concerned about the cosmetic appearance.

Questions

1. What is the diagnosis?
2. What is the systemic association with this dermatosis?
3. How are these lesions managed?

These cutaneous lesions are characteristic of necrobiosis lipoidica (NL). They are typically multiple, bilateral, and located on the lower legs, most commonly pretibially[1] and occasionally on the thighs, ankles and feet, but rarely on the trunk, upper limbs and scalp. Early lesions appear as rounded, dull red, symptom-less papules or plaques that progress slowly and indurate with central atrophy. The lesions have a shiny surface and a waxy, yellowish central area with prominent telangiectasias. The margins may carry comedone-like plugs. Koebner′s phenomenon occurs in some patients. The clinical and histopathological differential diagnoses include rheumatoid nodules, granuloma annulare, necrobiotic xanthogranuloma, sarcoidosis, morphea, stasis dermatitis, subacute nodular migratory panniculitis, erythema nodosum, erythema induratum, lichen sclerosus et atrophicus, tertiary syphilis, radiodermatitis, sclerosing lipogranuloma, and Hansen′s disease.[2]

An association with diabetes mellitus has been recognized for a long time. NL was originally termed dermatitis atrophicans lipoidica diabeatica (Oppenheim 1929) and later renamed necrobiosis lipoidica diabeticorum (Urbach 1932). In one large series, 111 of 171 patients (65%) with NL had diabetes mellitus at presentation;[3] it preceded the onset of diabetes in 15% of patients. Its prevalence is 0.3% in people with diabetes, is three times commoner in women, and occurs usually before 30 years of age. In our patient, Type 1 diabetes occurred two years prior to the first lesion. Her glycaemic control was poor and she had diabetic retinopathy as well.

The aetiology remains obscure, but is not a microangiopathy and not associated with glycaemic control or chronic diabetic complications. Antibody-mediated vasculitis and abnormalities of collagen are the other chief putative mechanisms. Histologically, NL occurs as palisading or pseudotuberculoid granulomatous lesions[1] consisting of foci of degenerate collagen bundles with a hyalinized appearance, surrounded by fibrosis, a diffuse infiltrate of histiocytes and a giant-cell granulomatous reaction. Capillary wall thickening and microvascular occlusion are often present.

Treatment of NL is unsatisfactory with cosmetic camouflage the preferred option. Regression of lesions does not correlate with improved glycaemic control. Topical or intralesional corticosteriods may improve early NL.[3] Psolarens and ultraviolet A (PUVA) therapy can improve patients not responsive to steriods.[4] Antiplatelet therapy with aspirin and dipyridamole has shown no benefit[5] but anecdotal reports with pentoxyfilline, tretinoin, nicotinic acid, topical tacrolimus, cyclosporine, and infliximab have all documented benefits. Excision and skin grafting may help some. Other complications include ulceration following trauma, occasionally infections and rarely squamous cell carcinoma.

REFERENCES

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