Acute Borrelia infection inducing an APMPPE-like picture
© The Author(s). 2016
Received: 24 April 2016
Accepted: 2 June 2016
Published: 13 June 2016
Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is an uncommon disorder of unknown etiology affecting the retina, the retinal pigment epithelium, and the choroid. Although several etiological factors have been suggested, none has been confirmed. We report a case of APMPPE associated with acute infection of Borreliosis. A 30-year-old man presented with a decrease in vision in the right eye of about 1-week duration. His visual acuity in the right eye was 6/36. Fundus exam revealed the presence of multiple placoid creamy retinal/subretinal lesions in the right eye. Fundus fluorescein angiography supported the diagnosis of APMPPE. Blood tests revealed the presence of concomitant acute Borreliosis infection, as confirmed by IgM. The patient received oral prednisone therapy and amoxicillin. Six weeks later, the visual acuity returned to 6/6, and the patient was symptom free. Borreliosis can have several manifestations in the eye. One of the less common presentations is an APMPPE-like picture. The clinician should suspect acute Borreliosis infection in patients presenting with APMPPE, especially when there is a history of a tick bite, when the patient has systemic symptoms, or when living in/visiting endemic areas. This may help in the prompt management of APMPPE, avoiding complications due to the condition itself, or systemic involvement secondary to the Borreliosis infection.
KeywordsAPMPPE—acute posterior multifocal placoid pigment epitheliopathy Lyme disease—Borreliosis Borrelia
Borreliosis (Lyme disease) is an infectious systemic disease caused by bacteria (a spirochete) of the Borrelia family. It is transmitted by the bite of a tick of the genus Ixodes [1, 2]. It may cause a systemic infection, involving the skin, joints, heart, nervous system, and sometimes the eye . Eye involvement may include conjunctivitis, uveitis, keratitis, optic neuritis, involvement of multiple cranial nerves, and pars planitis . In this case report, we describe a patient that presented with acute posterior multifocal placoid pigment epitheliopathy, and was found to have a concomitant acute Borreliosis infection as confirmed by IgM levels. Although a relation between APMPPE and Borreliosis had been previously suggested in the literature, this association has not been proven. In our case report, we explore this relation further.
Six weeks after taking the treatment, the patient’s visual acuity improved to 6/6 in both eyes and the retinal lesions completely resolved. The patient tolerated the treatment well with no side effects.
Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a rare inflammatory condition of unknown etiology affecting the retina, the retinal pigment epithelium, and the choroid [5, 6]. It was first described by Gass in 1968. It was hypothesized back then that APMPPE may be caused by an infectious agent. However, this could not be proven. The pathogenesis till now is still not absolutely clear, but it is speculated to involve inflammation and ischemic changes. For the diagnoses to be made, a high index of suspicion is needed, based on the clinical picture mainly and supported by fundus fluorescein angiography findings. Although a relation between APMPPE and Borreliosis had been suggested in the literature, this association was never confirmed. And since APMPPE is an uncommon condition, no major study was done to explore its association with Borreliosis. The biggest study that we were able to find was by Wolf et al. in 1992 and it included 18 patients. None of the patients with APMPPE in this study had positive antibodies against Borrelia burgdorferi . As a result, the investigators concluded that they were not able to establish an association between these two conditions. However, a few case reports were published since then that showed that this association may actually be true. We found a case report by Augsten et al. in 2009  and another in early 2015 by Wang et al.  that suspected this association. Our case report, to our knowledge, will be the second from the year 2015 to be published in the literature, and the fifth to be reported from Germany [10–12]. Furthermore, it seems curious to us that most of the above mentioned reports that suggest a link between the two diseases come from Europe, and that there are multiple bacterial species causing Lyme disease in Europe (Borrelia afzelli, Borrelia garinii, and B. burgdorferi), while in America the disease is mainly caused by one species only (B. burgdorferi) [13, 14]. Whether this observation is a coincidence or not is yet to be determined.
Our case report is not the first to explore the relation between Borreliosis and acute posterior multifocal placoid pigment epitheliopathy. Although these reports are few in number, they do suggest a possible association that is worth further investigation. If such an association could be proven, even in a minority of patients, this would confirm the importance of setting new recommendations and guidelines that may help the clinicians in the management and treatment of patients who suffer from APMPPE and/or Borreliosis. We would also like to emphasize on the importance of suspecting acute Borreliosis infection in patients presenting with APMPPE, especially if there is history of tick bite, presence of systemic symptoms, or living in/visiting endemic areas.
Dr Michael Mueller made the initial diagnosis of the patient and follow-up. Dr Ninel Kinekstul withdrew blood samples from the patient and provided follow-up for the patient. Mr. Franco Lopez performed the OCT and fluorescein angiography for the patient.
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