Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) was described by Gass1 in 1968 and is characterized by acute onset of multiple pale yellow-white placoid lesions at the level of the retinal pigment epithelium (RPE).123456 Typically it is bilateral and resolves spontaneously within 2 to 3 weeks leaving discrete pigment epithelial scars. Case reports have appeared in literature with RPE lesions characteristic of APMPPE associated with other atypical features such as serous detachments of the posterior retina, retinal vasculitis and papillitis,23 unilaterality34 and appeared to be intermediate between APMPPE and Harada's disease.5 The purpose of the present communication is to report four cases of APMPPE with associated atypical features, who responded well to oral prednisolone (1 mg/kg body weight tapered by 10 mg every week over 2 months), suggesting a role of corticosteroids in atypical cases.
The table shows the clinical details of the four patients with atypical APMPPE. Two patients presented with headache (cases no.1, and 2). Both eyes were involved in one patient (case 1) while the condition was unilateral in the remaining three patients. Gross reduction of vision was the chief complaint in all the patients. Relative afferent pupillary defect was present in all the involved eyes. There was associated lid edema and ptosis in one patient (case no.3). Focal vasculitis was present in right eye of case no.1 and involved eye of case no. 2. Serous retinal detachment and increased choroidal thickness (detected by ultrasonography) were present in cases 2, 3 and 4. There was vitritis in case no. 3.
The following were the atypical presenting features in these four patients-: case 1 - multiple, discrete, placoid, yellow-white lesions in the posterior pole at the level of the RPE in the right eye and a single lesion in the left eye; case 2- multiple bullae of serous retinal detachments at the posterior pole, and an area of RPE atrophy and hyperpigmentation corresponding to an old chorioretinal scar in the temporal retina in the left eye. Cases 3 and 4 had retinal lesions similar to case no. 2.
Fluorescein angiography showed early hypofluorescence and late hyperfluorescence of the lesions [Figures 1 and 2]. All patients were put on oral corticosteroids in tapering doses. (1 mg/Kg body weight tapered by 10 mg every week over two months).
In all eyes vision improved and headache was relieved in cases 1 and 2. There was recurrence of symptoms in cases 2 and 3 following withdrawal of corticosteroids, and responded well to reinstitution of corticosteroids. There was resolution of serous retinal detachments as well following treatment. The patients have remained stable over one year of follow up.
The defective visual acuity at presentation, in all the four cases could be attributed to papillitis and additionally foveal neurosensory retinal detachment in cases 2, 3, and 4. As suggested by Kirkham et al,2 the presence of papillitis in APMPPE probably reflects involvement of the choroidal supply to the optic nerve head by the disease process. The presence of optic nerve involvement suggests a poor prognosis and is a probable indication for systemic corticosteroids.
Unilateral involvement was another atypical feature. All the cases except case 1, had unilateral involvement in contrast to the classic bilateral occurrence of APMPPE. Whether the unilaterality is by itself a poor prognostic factor has to be ascertained by a longer follow-up of a larger number of patients.
There has been a lot of debate on the etiopathogenesis of APMPPE. Gass1 suggested an acute cellular response of the RPE and choroid to an unknown (possibly viral) agent. This is supported by the occurrence of a prodromal flu-like illness in over 50% of cases of APMPPE. Van Buskirk and others6 postulated focal choroidal vasculopathy as the etiological factor. Retinal vasculitis has been observed in some cases of APMPPE.2 We believe that fluorescein angiographic demonstration of segmental retinal vasculitis in cases 1 and 2 points to the possible involvement of the retinal vascular endothelium along with the choroidal vasculature in the pathogenesis of this disorder. The ocular involvement could be secondary to a local hypersensitivity to a toxin as seen in case 3 where there was a rapid onset of symptoms following an insect bite. The presence of markedly elevated Electrolyte Sedimentation Rate levels in cases 1 and 3 and positive Antinuclear Antibodies in case 1 suggest an immunologic/inflammatory etiology of APMPPE.
All the four patients had resolution of their ocular and in selected cases, systemic symptoms with corticosteroids. We hence feel that the institution of corticosteroids was justified in our patients.
Cases 2, 3 and 4 had serous detachments of the neurosensory retina and increased thickness of the choroid on ultrasonography. Curiously in both cases 2 and 3 recurrence was associated with an increase in choroidal thickness. We hence suggest that choroidal thickness, as measured by ultrasonography, may be used as a marker for recurrence in atypical cases of APMPPE.
Neurological manifestations of APMPPE such as cerebrospinal fluid pleocytosis and headaches have been well stressed in literature.7 The presence of headache in cases 1 and 2 could reflect a co-existent cerebral vasculitis.8 We would have considered further neurologic evaluation (Cerebrospinal Fluid analysis, Magnetic ResonanceImaging and cerebral angiogram) in cases 1 and 2, had the headache been persistent.
In conclusion, the atypical features of unilaterality, serous detachments of the neurosensory retina, retinal vasculitis and papillitis seen in these four cases are suggestive of a vascular disturbance in APMPPE. This vascular disturbance is in all probabilities a generalized autoimmune or inflammatory process which mandates the use of systemic corticosteroids. We need a longer period of observation and more number of patients to substantiate these initial observations.
1. Gass JD. Acute posterior multifocal placoid pigment epitheliopathy Arch Ophthalmol. 1968;80:177–85
2. Kirkham TH, Ffytche TJ, Sanders MD. Placoid pigment epitheliopathy with retinal vasculitis
and papillitis Br J Ophthalmol. 1972;56:875–80
3. Savino PJ, Weinberg RJ, Yassin JE, Pilkerton AR. Diverse manifestations of acute multifocal placoid pigment epitheliopathy Am J Ophthalmol. 1974;77:659–62
4. Pagliarini S, Piguet B, Ffytche TJ, Bird AC. Foveal involvement and lack of visual recovery in APMPPE associated with uncommon features Eye. 1995;9:42–7
5. Wright BE, Bird AC, Hamilton AM. Placoid pigment epitheliopathy and Harada's disease Br J Ophthalmol. 1978;62:609–21
6. Van Buskirk EM, Lessell S, Friedman E. Pigmentary epitheliopathy and erythema nodosum Arch Ophthalmol. 1971;85:369–72
7. Bullock JD, Fletcher RL. Cerebrospinal fluid abnormalities in acute posterior multifocal placoid pigment epitheliopathy Am J Ophthalmol. 1977;84:45–9
8. Comu S, Verstreten T, Rinkoff JS, Busis NA. Neurological manifestations of Acute posterior multifocal placoid pigment epitheliopathy Stroke. 1996;27:996–1001