Anetoderma. A possible new risk factor for subretinal neovascularization

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Editor, Anetoderma is a rare disease of the elastic connective tissue which is characterized clinically by multiple 5–25 mm-diameter round atrophic papules that bulge outwards from the skin of the trunk, the upper part of extremities and neck. At palpation, the lesions are flaccid and herniate inwards. Histopathologically, there is fragmentation of elastic fibres and loss of elastic tissue in the dermis. The aetiology of anetoderma is unknown, but the disease has been related to antiphospholipid syndrome, systemic lupus erythematosus (Venencie et al. 1984) and infections such as borreliosis (Bauer et al. 2003). In rare cases, the disease has been shown to be inherited or idiopathic. In the eye, anetoderma has been shown to be related to keratoconus, blue sclerae, pseudotumor orbitae and cataract (Brenner et al. 1977, Nuñez et al. 1995), whereas no studies have reported a relation between anetoderma and diseases in the posterior segment of the eye. A 27-year-old otherwise healthy woman with previous normal vision was referred with a 2 weeks history of metamorphopsia and blurred vision on the left eye. There was no history of trauma, choroiditis, or other known risk factors for the development of subretinal neovascularization, but anetoderma (Schweninger–Buzzy type) confirmed by skin biopsy had been diagnosed at the age of 12 years. Laboratory examinations for antinuclear antibodies (ANA), cardiolipin antibody and serological tests for Borrelia, syphilis and HIV were negative. The patient was subjected to a routine clinical examination including measurement of visual acuity, slit lamp examination, optical coherence tomography scanning and fluorescein angiography. The clinical examination showed VAD = 1.2 and VAS = 0.1 and both eyes had emmetropia. All other examinations on the right eye were normal. On the left eye, ophthalmoscopy showed central retinal oedema with haemorrhages and exudates. Optical coherence tomography (OCT) scanning of the left eye showed intra-retinal oedema with a central retinal thickness of 452 microns. Fluorescein angiography revealed a classical subfoveal neovascularization (Fig. 1). After three Intra-vitreal injections of ranibizumab on the left eye with 1 month intervals, the central retinal thickness was reduced to 214 microns and visual acuity had increased to 0.8. An early phase angiography of the normal right eye and the left eye with a subfoveal neovascularization. Subretinal neovascularization is a well documented complication to connective tissue diseases such as pseudoxanthoma elasticum and Ehlers Danlos’ syndrome and may be related to discontinuities or breaks in Bruch’s membrane that forms a barrier for growth of choroidal vessels under normal conditions (Georgalas et al. 2009). The present case report suggests anetoderma as a possible new risk factor for the development of subretinal neovascularization. A more detailed elucidation of the changes in elastic tissue in anetoderma and other connective tissue diseases may help understanding the pathophysiology of subretinal neovascularization in general.