This 55-year-old female is in good general health. She is not a diabetic, and is a smoker. Current medications include Dilantin, glucosamine, and vitamin E. In the past she has used New Zealand honey under occlusion, plus various other herbal remedies. The left upper shin had two small clustered sclerotic areas with small ulcerated areas in the center (see Figure 1). The right shin had a few discolored patchy looking areas (see Figure 2). The patient also had a venous insufficiency to her lower legs.
Small clustered sclerotic areas with small ulcerated areas in the center.
The right shin contained a few discolored patchy looking areas.
Patients with necrobiosis lipoidica diabeticorum may or may not be diabetic. The affected areas usually occur on the lower legs, but can occur elsewhere. This rare skin disease belongs to the idiopathic, cutaneous, palisading granulomatous dermatides associated with degradation of collagen which leads to skin atrophy.
Idiopathic: a spontaneous disease without a cause.
Palisading: a layer of parallel elongated cells below the epidermis resembling a fence of stakes.
Granulomatous: pea-sized nodules crusted with vascular elements. May follow minor trauma or secondary infection.
Degradation: breaking down.
Necrobiosis lipoidica diabeticorum is three times more common in women than in men. It is usually located on the shins and starts as erythematous papules over the pretibial areas and may enlarge slowly. It is generally asymptomatic. The lesions are usually well-demarcated, shiny, atrophic, yellowish or red brown, and can be bilateral. Differential diagnosis can include granuloma annulare, morphea, stasis dermatitis, cellulitis, sarcoidosis, lichen sclerosis, trauma, tertiary syphilis, radiodermatitis, sclerosing lipogranuloma, and Hansen's disease.
Treatments are usually only marginally effective. Lesions resolve spontaneously in 13% to 19% of cases. Residual scarring, atrophy, cosmetic disfigurement, central atrophy, and ulceration can occur.
This patient was offered and instructed on PUVA soaks. She was required to come to the phototherapy center twice a week with 48 hours in between treatments. The need for consistency was emphasized. The phototherapy center uses a 1 to 4 parts oxsoralen mixture. Six pills per 60 liters of water was used to soak her lower legs for 15 minutes in a large tub of water. The patient was given a great deal of emotional and moral support to encourage regular treatment and compliance. A total of 29 treatments and 96.5 joules of UVA light were given over several months. PUVA may be useful through an immunomodulatory mechanism or an inhibiting effect on DNA synthesis. The inflamed edge seems to respond better than the ulcerated center, so PUVA may be more effective in earlier lesions. An antibiotic cream was used on the ulcerated area plus Dermovate bid and moisturizers for dry skin. The patient was also instructed to obtain support stockings for venous insufficiency with instructions on how to put them on and when to wear them. The patient felt the affected area had improved but was worried about the side effects of light treatment. Light was discontinued and the patient was discharged to her dermatologist for followup.
Treatments can include steroid injections, tretinoin, vitamin A, topical steroids, cyclosporine, excision of the area, skin grafting, laser topical PUVA, anti-thrombolytic agents, heparin, and anti-platelet agents.
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