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Acute multifocal posterior placoid pigment epithelio pathy (AMPPPE) is a disorder of otherwise healthy young adults. A possible thrombotic process involving the choroid has been suggested in the pathogenesis of this condition. We report a case of AMPPPE and anticardiolipin antibodies (aCL).
A 32 year old woman presented because of sudden onset of bilateral decrease in visual acuity. Her past medical history was negative. One week before the decrease in vision, the patient had been complaining of headaches associated with fever, chills, and a rash on her neck and lower extremities.
The initial ocular examination showed a visual acuity of 20/200 in the right eye and 20/30 in the left eye. Fundus examination disclosed bilateral multiple yellow-white placoid lesions in the posterior pole at the level of the retinal pigment epithelium (RPE). Serous detachment of the right macula was also noted. Fluorescein angiography showed early hypofluorescence of the lesions with staining in the late phases of the angiogram. Based on the clinical and fluorescein angiogram findings, the diagnosis of AMPPPE was made.
Physical examination showed a temperature of 38.5°C, tender posterior cervical lymphadenopathy, as well as skin lesions affecting both legs. Biopsy of the lesions was compatible with erythema nodosum. Extensive fever investigation was negative. The erythrocyte sedimentation rate was 69 mm/1st h, and IgG aCL was 78 GPL (normal <15.0 GPL); IgM aCL was negative.
Over a period of two weeks, the fever resolved and the patient’s vision started to improve without any treatment. The fundus lesions disappeared leaving irregular RPE pigmentation. One month later her vision had recovered to 20/30 in the right eye and 20/25 in the left eye. There was total resolution of the serous detachment in the right eye. Two, four, and six months after presentation, the IgG aCL were 63, 57, and 52 GPL, respectively. One year later, the aCL titres were normal. The eye and systemic examination remained normal throughout this period.
AMPPPE has been associated with a wide variety of disorders, including viral infections (adenovirus), streptococcal infections, vasculitides (cerebral vasculitis and Wegener’s granulomatosis), post-hepatitis B vaccination, Lyme disease, tuberculosis, and sarcoidosis.1–3 Few reports have suggested an immunological mechanism.
The pathogenesis of AMPPPE remains obscure.4 AMPPPE has been associated with retinal vascular occlusive disease. Charteris et al reported a case of AMPPPE complicated by a central retinal vein occlusion.5 The occurrence of these two conditions at the same time may imply that AMPPPE is part of a wider thrombotic disease. Furthermore, the presence of a serous macular detachment further supports injury to the choriocapillaris and RPE. Serous macular and retinal detachment are often seen in occlusive diseases of the choriocapillaris (for example, toxaemia of pregnancy, disseminated intravascular coagulopathy).6
Antiphospholipid antibodies (aPL) have been reported in various ophthalmological conditions, including amaurosis fugax, retinal arterial and venous occlusion, and transient diplopia as well as in recurrent ischaemic optic neuropathy.7 They have not been previously described in association with AMPPPE. The aPL have been frequently detected in patients with various infectious diseases.8 Although aPL associated with infections were initially thought to be non-pathogenic and not associated with thrombotic complications,8 recent data uncovered a possible pathogenic role for these antibodies in precipitating thrombosis.8 Lupus anticoagulant or aCL, or both, were associated with a number of viral infections like hepatitis C virus, human immunodeficiency virus, cytomegalovirus, varicella zoster, Epstein B virus, adenovirus, and parvovirus B. In many instances the presence of these antibodies was associated with thrombosis.8
The presentation and course of our patient suggest a possible role for aCL in the pathogenesis of AMPPPE.
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