Treatment of Ulcerated Necrobiosis Lipoidica With Intravenous Immunoglobulin and Methylprednisolone
February 2012 | Volume 11 | Issue 2 | Case Report | 256 | Copyright © 2012
The authors report a female patient with recalcitrant ulcerated necrobiosis lipoidica (NL) that was resistant to numerous systemic agents and who responded to treatment with intravenous immunoglobulin (IVIG), leading to resolution of the ulcerated areas for several months. Subsequent treatment with two further courses of IVIG was less effective, but a course of intravenous methylprednisolone led to regression of the lesions. As well as briefly reviewing the literature on treatments used to treat ulcerated NL, we outline the pathological mechanisms thought to be involved in the condition and how the modes of action of IVIG might explain its apparent efficacy in this case. As far as we are aware, the response of ulcerated NL to IVIG or methylprednisolone has not been reported previously, although other systemic preparations of corticosteroids have been used.
J Drugs Dermatol. 2012;11(2):256-259.
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Necrobiosis lipoidica (NL) is a chronic granulomatous dermatitis that usually presents as waxy yellow atrophic plaques on the shins, although lesions can develop elsewhere. Two-thirds of patients have associated diabetes mellitus. In many patients, NL causes only cosmetic problems although painful, ulcerated lesions may arise, and these can be complicated by squamous cell carcinoma. Severe, ulcerated NL is often resistant to treatment.1 To our knowledge, the use of intravenous immunoglobulin (IVIG) in the treatment of NL has not been reported previously.
A 40-year-old female patient was referred in 1991 with a three-month history of two erythematous plaques on the left shin. The lesions were asymptomatic but had enlarged to approximately 2 cm in diameter. She had a 20-year history of type I diabetes mellitus. Skin biopsy confirmed the clinical diagnosis of necrobiosis lipoidica (NL), and the lesions were treated successfully with potent topical corticosteroids. However, over the following 10 years, a single, slowly-enlarging plaque developed on the left shin, together with new lesions on the right shin.
In 2002, the plaque on the left shin, now measuring 15x8 cm, was traumatized and became painful.Topical and intralesional corticosteroids, 0.1% tacrolimus ointment under occlusive dressings and an 18-week course of twice-weekly topical Psoralen and ultraviolet A (PUVA) all brought no improvement.
By 2005, the plaque on the left shin had become ulcerated, painful, sloughy, and infected, requiring treatment with several courses of oral antibiotics. Oral pentoxyfilline was considered but was contraindicated due to proliferative retinopathy and previous history of retinal haemorrhages. Oral hydroxychloroquine was commenced but had to be discontinued owing development of urticaria and angioedema.
The ulcerated areas of NL became infected with Pseudomonas species, resistant to oral antibiotics. The patient was admitted for treatment with intravenous meropenem, plus oral prednisolone 40 mg once daily, nicotinamide 500 mg three times daily, and lymecycline 408 mg once daily. Larvae were administered to the sloughy areas. However, the lesions remained painful, ulcerated, and highly debilitating (Figure 1). The patient's Dermatology Life Quality Index (DLQI) at this time was 23/30. Treatment with ciclosporin was considered but declined by the patient, who by this time had developed stage III chronic kidney disease secondary to her diabetes.
Given the intractable nature of the NL and lack of therapeutic options, a 5-day trial of intravenous immunoglobulin (IVIG) 2 g/kg