Necrobiosis lipoidica and inflammatory eye disease

Skin and ocular inflammation can co-exist in several inflammatory conditions such as rheumatoid arthritis, sarcoidosis, Wegener’s granulomatosis and polyarteritis nodosa. We report a case of non-diabetic necrobiosis lipoidica in whom periods of skin inflammation were associated with exacerbations of ocular inflammation in the form of intermediate uveitis and retinal vasculitis over 20 years. In July 1988, a 43-year-old Caucasian woman presented with blurred vision and floaters with a three-year history of recurrent iritis in both eyes. Visual acuities were 6/5 in the right eye and 6/6 in the left eye. As she had bilateral inflammatory cells in the anterior chamber and a vitreitis with inferior vitreous aggregates (‘snowballs’) in both eyes, she was diagnosed with bilateral intermediate uveitis, which over a 9-month period led to cytoid macular oedema in the right eye (visual acuity 6/6). Over the next 24 moths bilateral obliterative peripheral retinal vasculitis occurred, though visual acuity remained 6/9 and 6/6 in right and left eye. Inflammatory markers, infective screen, serumACE and chest X-ray were all within normal limits. Concurrently she had developed atrophic plaques on the skin of both shins associated with impaired light-touch sensation on the right leg. Her past medical history was significant for granuloma annulare on her hands at age 24 years. Fasting blood glucose and HbA1C were normal and Doppler ultrasound studies of the lower limbs excluded arteriovenous pathology. A skin biopsy confirmed the diagnosis of necrobiosis lipoidica. The skin plaques intermittently ulcerated in the years following diagnosis, coinciding with exacerbations of her ocular signs on each occasion. Cutaneous treatments over a 20-year period ranged from intralesional triamcinolone (10 mg/mL) to oral nicotinamide 500 mg tds, clofazime 200 mg od, cyclophosphamide 100 mg bd (3 mgkg) and topical GM-CSF, each variably effective. Her ocular inflammation required only topical corticosteroids, until 10 years after first presentation when the retinal vasculitis in the left eye (Figure 1a) led to peripheral retinal neovascularisation, treated successfully with laser panretinal photocoagulation. Visual acuities were 6/9 in the right eye and 6/6 in the left eye. Both ocular and cutaneous inflammation remained controlled for another 9 years, when she had another concurrent recurrence of both bilateral intermediate uveitis and necrobiosis lipoidica (Figure 1b). Repeat skin biopsy (Figure 1c) revealed normal epidermis but the dermis showed a moderate, sharply demarcated predominantly plasmacytic perivascular infiltrate with foci of multinucleated giant cells in the deep dermis. The deeper part of the sample showed necrobiotic collagen. There was a small focus of fat necrosis within the collagen and the medium-size deep dermal artery showed moderately thickened media. Mild positive staining for complement C3 was seen in the endothelium of the small dermal vessels on immunohistochemical staining but no obvious DECLARATIONS