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Arch Ophthalmol. 2004;122:1881-1882.

The white spot syndromes are a group of idiopathic inflammatory diseases of the retina characterized by visual loss in association with areas of retinal whitening. This category includes diseases such as multifocal choroiditis (MFC), punctate inner choroidopathy, multiple evanescent white dot syndrome (MEWDS), serpiginous choroiditis, and acute posterior multifocal placoid pigment epitheliopathy (APMPPE). To our knowledge, no definite infectious or immune etiology has been proved for these various entities.¹ There have been reports of 2 of these entities occurring in the same patient (acute macular neuroretinopathy and MEWDS² and MFC and MEWDS³). Patients have been described as having overlapping features of these various conditions, eg, MEWDS and MFC. We describe a patient who at age 18 years showed findings consistent with APMPPE, with visual loss in both eyes. This resolved with a return of vision to 20/20 OU. Sixteen years later, he developed new symptoms and exhibited lesions of MFC. The old APMPPE lesions remained unchanged.

Report of a Case
An 18-year-old man sought care because of headaches and bilateral central scotomas of 1 week’s duration. He reported having had an upper respiratory tract infection 1 month earlier. Visual acuity was 20/80 OD and 20/30 OS. Ophthalmoscopy showed scattered active foci of whitening of the outer retina in both eyes (Figure 1A and B). Fluorescein angiography showed hypofluorescence during the early phases of the angiogram with late staining of the lesions (Figure 1C and D). A diagnosis of APMPPE was made, but no treatment was given. During the next 3 months, his symptoms gradually resolved and visual acuity returned to 20/20 OU. The patient had no additional eye symptoms until 16 years later, when he noted the onset of a temporal scotoma and peripheral shimmering in the left eye. Best corrected visual acuity was 20/25 OD and 20/30 OS. The vitreous body was clear. The macular area showed the old lesions of APMPPE (largely unchanged and without the development of significant atrophy). However, both eyes showed atrophic punched-out chorioretinal scars (Figure 2) in the macula, midperiphery, and far periphery. The appearance of these scars and the patient’s symptoms were consistent with a diagnosis of MFC.

View larger version (106K): [in this window] [in a new window] Figure 1. Ophthalmoscopic and fluorescein angiographic images of the patient at age 18 years. Fundus photographs of the right (A) and left (B) eyes, demonstrating multiple cream-colored lesions beneath the retina. On fluorescein angiography of the left eye, these lesions block early (C) and stain late (D), consistent with acute posterior multifocal placoid pigment epitheliopathy.

View larger version (39K): [in this window] [in a new window] Figure 2. Ophthalmoscopic and fluorescein angiographic images of the same patient 16 years later. Fundus photographs of the right (A) and left (B) eyes, demonstrating multiple peripapillary and midperipheral punched-out scars consistent with multifocal choroiditis and the residual different scars from the acute posterior multifocal placoid pigment epitheliopathy 16 years earlier. C, Fluorescein angiography of the left eye, demonstrating the window defects and pigment mottling of lesions of multifocal choroiditis.

Comment
Gass⁴ has proposed that many of these white spot syndromes, including MEWDS, punctate inner choroidopathy, and MFC, as well as acute zonal ocult outer retinopathy, have common characteristics and may be part of the spectrum of a single disease. He has suggested an infectious cause, in which an unknown virus enters the retina from the peripapillary area or perhaps the ora serrata.⁵ Jampol and Becker¹ have suggested that these entities are distinct inflammatory diseases, although overlapping cases and the occurrence of 2 of the entities in a single patient may occur. They concurred with the common genetic hypothesis that autoimmune inflammatory disease may explain these findings.¹ These patients may have genetic loci that predispose them to immune dysregulation and ocular and systemic autoimmune disease. This may explain the potential for 2 entities occurring in a single patient or overlapping symptoms. The case presented herein is a demonstration of unusual concordance of 2 rare diseases, APMPPE and MFC, in the same patient, separated by 16 years. Although the patient’s initial appearance at age 18 years was highly suggestive of APMPPE, this may have been an atypical presentation of MFC. However, the clinical course was most consistent with APMPPE. The fact that the APMPPE lesions, even 16 years later, did not resemble the MFC lesions supports our conclusion that 2 distinct entities occurred in this patient. This occurrence is consistent with the common genetic hypothesis of a genetic predisposition to autoimmune diseases, which allowed both diseases to occur in a single individual.

AUTHOR INFORMATION
Correspondence: Dr Jampol, 645 N Michigan Ave, Suite 440, Chicago, IL 60611 (l-jampol{at}northwestern.edu).

Financial Disclosure: None.

Funding/Support: This study was supported in part by an unrestricted grant from Research to Prevent Blindness, Inc, New York, NY.

Jeevan R. Mathura, Jr, MD; Lee M. Jampol, MD; Mark J. Daily, MD

REFERENCES
1. Jampol LM, Becker KG. White spot syndromes of the retina: a hypothesis based on the common genetic hypothesis of autoimmune/inflammatory disease. Am J Ophthalmol. 2003;135:376-379. FULL TEXT | PUBMED 2. Gass JD, Hamed LM. Acute macular neuroretinopathy and multiple evanescent white dot syndrome occurring in the same patients. Arch Ophthalmol. 1989;107:189-193. ABSTRACT 3. Bryan RG, Freund KB, Yannuzzi LA, Spaide RF, Huang SJ, Costa DL. Multiple evanescent white dot syndrome in patients with multifocal choroiditis. Retina. 2002;22:317-322. FULL TEXT | PUBMED 4. Gass JD. Overlap among acute idiopathic blind spot enlargement syndrome and other conditions. Arch Ophthalmol. 2001;119:1729-1731. FREE FULL TEXT 5. Gass JD. Are acute zonal occult outer retinopathy and the white spot syndromes (AZOOR complex) specific autoimmune diseases [comment]? Am J Ophthalmol. 2003;135:380-381. FULL TEXT | PUBMED

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