Indian Journal of Ophthalmology

ORIGINAL ARTICLE
Year
: 1982  |  Volume : 30  |  Issue : 1  |  Page : 15--18

Acute posterior multifocal placoid pigment epitheliopathy


SP Dhir, IS Jain, DN Gangwar, AV Joshi 
 Department of Ophthalmology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Correspondence Address:
S P Dhir
Department of Ophthalmology, Postgraduate Institute of Medical Education and Research, Chandigarh
India




How to cite this article:
Dhir S P, Jain I S, Gangwar D N, Joshi A V. Acute posterior multifocal placoid pigment epitheliopathy .Indian J Ophthalmol 1982;30:15-18


How to cite this URL:
Dhir S P, Jain I S, Gangwar D N, Joshi A V. Acute posterior multifocal placoid pigment epitheliopathy . Indian J Ophthalmol [serial online] 1982 [cited 2021 Jun 17 ];30:15-18
Available from: https://www.ijo.in/text.asp?1982/30/1/15/27909


Full Text

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) was described for the first time by Gass [1]. Since then many reports on this disease have appeared in the literature [2],[3],[4],[5],[6],[7],[8]. It is a newly recognised disorder of the fundus. It is characterised by acute onset, usually a quiet anterior segment and fundus showing flat, yellow-white (grey) multiple lesions at the level of retinal pigment epithelium at the posterior pole. It is rela­tively a benign disorder. The fundus lesions resolve spontaneously, leaving a residual defect in the pigment epithelium and vision is affected only when the lesions involve the macula.

We report six cases of APMPPE examined in the last 3 years to record the occurrence of this disease entity in India.

 CASE REPORTS



1. A.P. 18 year Male (E-95915) was seen with diminution of vision in both eyes for the last 4 days. On examination his visual acuity was reduced in both eyes to 6/12. Anterior segments were quiet. Fundi showed clear media. Discs and periphery of the fundi were normal. Macular area of both eyes showed multiple, greyish, hypopigmented, well demarcated plaque like lesions. These lesions were not raised from the surface and the intervening retina was normal. [Figure 1].

All investigations for uveitis were normal. Fluorescein angiography revealed multiple patches of window defects and a few patches blocking the choroidal fluorescence [Figure 2]. He was diagnosed as a case of APMPPE both eyes.

He received 40 mg daily systemic steroids which were gradually tapered. His visual acuity improved to 6/6 in both eyes. After two months he was seen with diminution of vision in Right eye again. On examination his visual acuity was reduced in R/E to 6/36. Anterior segment was quiet. Fundus examination revealed clear media and macular area showed new multiple plaque like lesions. Repeat fluorescein angiography showed similar changes as seen previously. He was again put on 30 mg daily systemic steroids which were gradually tapered. His visual acuity has improved to 6/9 in RE and remained 6/6 in LE.

2. K.S. 32 female (J-105784) presented with diminished vision from both eyes of short duration. Examination of RE revealed visual acuity of 6/60 with white and quiet anterior segment. Fundus examination revealed a clear media with normal optic disc. Multiple greyish lesions were seen at the posterior pole. These lesions were flat and deeper to the retinal vessels. Fluorescein angiography revealed a large number of irregular lesions, some of them blocking the choroidal fluorescence and others showing staining of the edges [Figure 3]. Examination of left eye revealed visual acuity of 6/6 with iris atrophy and a fine posterior synechia at 6'o' clock position. Early lenti­cular changes were noted under the posterior capsule and media was some what hazy with cells in the vitreous. Optic disc was normal. Patches similar to right eye were seen at the posterior pole and some macular oedema was also seen. Fluorescein angiographic appearance was also similar to that of RE. X-ray chest showed consolidation of middle zone on left side. Relevant investigations revealed no other abnormality. She received intra venous calcium gluconate systemic and topical steroids. Her visual acuity in RE improved by one line only.

3. C.P.S. 27 year male (E-91110) was seen with diminition of vision from LE for the last 5 days. On examination RE had visual acuity of 6/12 with quiet anterior segment. Fundus showed a clear media and normal optic disc. There were multiple greyish patches, with pigmented edges lying deeper to the reti­nal vessels in the central area. Periphery of the fundus was normal. LE had a visual acuity of 6/18 with quiet anterior segment. Fundus showed a clear media with a coma shaped dark mass with surrounding retinal oedema extending towards the disc. Isolated hypopigmented patches were seen near the inferior temporal vessels about 3 discs diameter from the disc. Fluorescein angiography revealed multiple patches of window defects and a few patches blocking the choroidal fluorescence. He was diagnosed as a case of acute multifocal placoid pigment epithelio­pathy both eyes. His visual acuity improved to 6/12 RE and 6/9 LE.

4. R.P. 49 year male (J-90452) was seen with complaint of blurring of vision from both eyes. On examination his visual acuity was reduced in both eyes to 6/36. Anterior seg­ments were quiet and media were clear. Discs and periphery of the fundi were normal. Macular area of both eyes showed greyish, hypopigmented plaque like lesions with well defined margins. Intervening retina was normal. Fluorescein angiography showed multiple lesions blocking the choroidal fluorescence in the early phase and staining of the edges of the lesions in the later phase. All relevant investigations pertaining to uveitis were non-contributory. Patient received daily 60 mg systemic steroids which were tapered gradually. His visual acuity in both eyes improved to 6/9 over a period of 20 days.

5. L.B. 28 years male (E-83616), was seen with the complaint of diminition of vision from RE for the last 7 days. On examination his visual acuity was reduced in RE to 6/36. Anterior segment was quiet and media was

clear. The disc and the periphery of the fundus were normal. Macular area showed multiple, punctate, hypopigmented greyish plaque like lesions with well defined margins, and radial striations. He received daily 40 mg systemic steroids which were tapered gradually. Investigations for uveitis revealed no abnor­mality. His visual acuity improved to 6/6 in RE.

6. M.M. 30 years male (x-97857) presen­ted with laziness of vision from right eye of one day duration. On examination his visual acuity in RE was reduced to 6/18. Anterior segment was white and quiet. Fundus exami­nation revealed a clear media with normal optic disc. Macular area showed multiple greyish lesions with scanty pigmentation lying deeper to the retinal vessels. Fluorescein angiography showed multiple lesions which blocked the choroidal fluorescence in the early phase and staining of the edges of the lesion in the later phase.

All relevant investigations for uveitis were negative. He received 60 mg systemic steroids daily which were gradually tapered. His visual acuity in RE improved to 6/6 over a period of 2 months.

 OBSERVATIONS



Total six patients were seen, five of them were male and one was female. The age of the patients ranged from 18 years to 49 years.

The presenting complaint was diminition of vision in 4 patients while haziness of vision and blurring of vision with central black spot in front of the eye in one each of the two patients.

All presented with acute onset. Five had involvement of both eyes while one patient had uniocular involvement. The visual acuity at the time of presentation ranged from 6/60 to 6/12 in the affected eye. Anterior segment changes were noted in only one patient (case No 2) in the form of iris atrophy, posterior synechia and posterior capsular cataract. Fundus examination revealed clear media in all 5 patients while one had cells in vitreous. Optic disc and periphery of the fundus were normal. Macular area showed multiple, hypopigmented, greyish plaque like lesions with well defined margins. Intervening retina was found to be normal. These lesions exhi­bited minimal marginal pigmentation. All the relevant investigations for uveitis were non-contributory. No systemic involvement was noted in 5 patients, while one patient showed consolidation of middle zone of left lung. Active treatment in the form of syste­mic steroids was given to all the 6 patients. Visual recovery started in 3 weeks time and recovery was good in general. One patient showed recurrence after 2 months. Though the initial attack was bilateral the recurrence was noted only in RE.

 DISCUSSION



As the patients usually present with rapid diminution of central vision with multi-focal, yellowish-white, placoid - lesions at the posterior pole, majority of these patients are diagnosed as choroiditis. Though these lesions appear to represent multiple focal areas of cellular infiltration of the choroid they differ from focal choroiditis in several aspects. The clear media with absence of cells in the vitreous in important to note. Secondly, the overlying pigment epithelium is diffusely involved, and no part of the lesion appears to be deep to, or extend laterally beyond, the area pigment epithelial involvement. The lesions are variable in size, flat or placoid. The overlying serous detachment of the retina is conspicuously absent. On follow-up these lesions show rapid resolution with permanent alteration in the pigment epithelium with minimal damage to the adjacent choroid and retina. Significant visual improvement sets it apart from choroiditis.

The aetiology of the lesion is unknown. The clinical picture represents an acute pig­ment epithelial cellular response perhaps to an infection or toxic agent. The choroidal vascular insufficiency or degeneration of the pigment epithelium are unlikely to be the causative factors. The disease occurs in both sexes. The investigations conducted for uveitis are not helpful. Fluorescein angio­graphy is very valuable in the diagnosis of this condition and present a characteristic picture. It also appears that the active treatment employed in these patients materially does not influence the course of the disease and recurr­ences may take place. Our observation indi­cate that this disease is an independent entity and only occasionally it may be associated or complicated by uveitis as in case No. 2. Awareness of this condition and its identifica­tion helps in differentiating it from early lesions of acute choroiditis with which it is generally confused. This condition runs a rapid course apparently unrelated to the therapy. In general the visual prognosis is good.

 SUMMARY



Clinical features and fluorescein angiogra­phic findings are presented in six patients of acute multifocal placoid pigment epithelio­pathy.

References

1Gass J.D.M., 1968, Arch. Ophthalmol 80: 177.
2Deutman, A.F., Oesterhnis, J.A., Boen-Tan T.N., Aandekerk, A.L., 1972, Br. J. Ophthalmol. 56: 863.
3Ryan, S.J. and Maumenee, A.E., 1972, Am.J. Ophthalmol, 74:1066.
4Fitzpatrick, P.J.; and Robertson. D.M., 1973, Arch. Ophthalmol. 89 :373.
5Azar, P. Gohd, R.S.: Wattman, D. and Gitter, K.A.; 1975, Am. J. Ophthalmol. 80: 1003.
6Sarino, P.J.: Weinberg, R,J.; Yassin, J.G. and Pinkerton, A.R., 1974, Am. J. Ophthalmol. 77 : 659.
7D-utman, A.F. and Frans Liou, 1977, Am. J. Ophthalmol. 84 : 652
8Hoit. W.S.; Regan, C.D. J. and Tremple, C 1976, Am. J. Ophthalmol. 81 : 403.