Acute Posterior Multifocal Placoid Pigment Epitheliopathy A Recurrence Richard A. Lewis, MD, Csaba L.

Martonyi

Acute posterior multifocal placoid pig¬ ment epitheliopathy recurred in a 23-yearold white woman. Forty-two days after the onset of symptoms and following the complete resolution of the initial bilateral lesions, a second crop of ophthalmoscopically and biomicroscopically similar lesions were seen in each eye. The cause of the disorder is unknown, and corticosteroid therapy is of doubtful benefit.

Acute posterior multifocal placoid pigment epitheliopathy re¬ curred in a 23-year-old woman. Al¬ though this unusual form of uveitis has been much discussed in recent lit¬ erature, we are unaware of the occur¬ rence of the disorder in any individ¬ ual on more than one occasion. In 1968, Gass1 described a syn¬ drome affecting three young women who developed rapid bilateral visual loss and characterized by the presence of multiple flat yellowish-white le¬ sions at the level of the retinal pig-

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publication April 29, 1974. From the Department of Ophthalmology, University of Michigan Medical Center, Ann Arbor, Mich. Reprint requests to Department of Ophthalmology, University of Michigan Medical Center, 1405 E Ann St, Ann Arbor, MI 48104 (Dr. Submitted for

Lewis).

ment epithelium with subsequent mini¬ mal damage to the adjacent choroid

and retina. These lesions screened the choroidal fluorescence during the ini¬ tial transit of dye and became hyperfluorescent during subsequent phases of fluorescein angiography. Sponta¬ neous resolution of the lesions oc¬ curred within a few weeks, and visual recovery was usually good, although

occasionally incomplete. Scattered areas of depigmentation and fine to coarse clumping of pigment remained

after resolution of the acute subretinal lesions. Extensive systemic examinations and blood studies failed to identify any specific etiologic factors. In ac¬ the cause was un¬ knowledging that known, Gass1 '' proposed the descrip¬ tive name "acute posterior multifocal

placoid pigment epitheliopathy." Schlaegel' and Maumenee4 casually reported other patients with this con¬

dition; van Buskirk et al5 described a patient with acute pigment epitheli¬ opathy associated with erythema nodosum. More recently, larger series, including many males, have been re¬ ported by Ryan and Maumenee," Fitzpatrick and Robertson,7 and Annesley et al.8

There has been no definite systemic illness associated with this condition, although an antecedent acute viral

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illness (either upper respiratory or gastrointestinal) has been reported"'8 in some patients. Its occurrence in a patient with systemic mycobacterial

infection has also been recorded." REPORT OF A CASE

A 23-year-old white female graduate student was evaluated at the University of Michigan Medical Center on June 18, 1971, because of a seven-day history of "spots" before her right eye and blurring of vision. Her general health was good, and she had experienced no antecedent illnesses. She had been taking an oral contraceptive (Ortho-Novum 1/50) for 18 months and had used a "tranquilizer" for a brief period in January and February 1971. At age five, she had undergone a tonsillectomy and adenoidectomy. Otherwise, her past medi¬ cal history and her family ocular and con¬ stitutional history were unremarkable. Ophthalmologic examination revealed a best-corrected visual acuity of 20/30 + 3 in the right eye and 20/20 in the left eye. Re¬ sults of the external, motility, and biomicroscopic examinations were normal. In¬ traocular tensions by applanation were 13 mm Hg in the right eye and 15 mm Hg in the left eye. Ophthalmoscopic examination of the right eye showed multiple, discrete, generally round and occasionally con¬ fluent, grey-yellow lesions at the posterior pole and in the midperiphery deep to the sensory retina (Fig 1). Some lesions showed early depigmentation centrally and fine pigment mottling near their slightly fuzzy margins. No subretinal hem-

Fig 1 .—Acute multifocal lesions at posterior pole of right eye June 18, 1971. Similar lesions were identified in left eye.

orrhage

or serous or

hemorrhagic detach¬

ment of the neuroepithelium or of the reti¬ nal pigment epithelium was noted. The

retina, the retinal vasculature, and the

overlying

vitreous were normal. The left eye showed similar posterior lesions. Color vision tested by the Hardy-RandRitter plates was normal in each eye. Cen¬ tral visual-field examination of each eye

showed multiple small round and ovoid paracentral scotomata to 2/1,000 white test objects corresponding to the subretinal le¬ sions. Intravenous fluorescence angiography of the right eye showed widespread discrete and confluent spots of hypofluorescence in the early retinal venous phase, correspond¬ ing to the creamy placoid lesions (Fig 2). The perimeter of each of these small dark islands became smaller and slightly fluo¬ rescent in the late retinal venous phase (Fig 3). In the recirculation phase, the le¬ sions began to stain softly with fluores¬ cein, and they remained visible at one hour

(Fig 4).

Four days later, fluorescence angiogra¬ phy performed on the left eye documented similar changes. Because of an initial impression of a multifocal "choroiditis," prednisone ther¬ apy (100 mg/day in divided doses) was ini¬ tiated orally. On June 23, 1971, the visual acuity was recorded as 20/40 in the right eye and 20/20 in the left eye. The biomicroscopic examination showed a quiet anterior

chamber in each eye. Occasional cells in the anterior vitreous of the right eye were ob¬ served. Ophthalmoscopy and visual-field

on

Fig 2.—Early

examination indicated no apparent change in the lesions. However, the decrease in vi¬ sual acuity and the identification of an inflammatory response in the anterior vitreous led to the administration of triamcinolone, 40 mg, below the Tenon space of the right eye. Results of general physical examination, chest roentgenogram, fasting blood glu¬ cose, two-hour postprandial glucose, hemo¬ globin, hematocrit, and urinalysis were normal. The white blood cell count, while on a

corticosteroids, was 22,000/cu mm with normal differential, although a "few

atypical lymphocytes" were seen. On July 2, 1971, the vision had improved to 20/20 in the right eye and 20/15 in the left eye. The lesions showed progressive clearing of the creamy-grey opacification and increased depigmentation and fine pigment clumping of the pigment epithe¬ lium. Administration of prednisone was ta¬ pered quickly and discontinued by July 16, 1971, when the visual acuity of 20/15 was recorded in each eye. The visual-field ex¬ amination showed that the scotomata had resolved almost completely although sev¬ eral, adjacent to fixation, could be plotted with 1/1,000 white and 2/1,000 red test objects. The pigment epithelial lesions showed mottled patches of clumped pig¬ ment.

On July 22, 1971, 42 onset of her

phase of fluorescein angiogram of right masking of normal choroidal fluorescence.

venous

eye shows multifocal

days after the initial

symptoms, the patient became

of "another scotoma" near fixation in her right eye and metamorphopsia in each eye. Although her vision was recorded as 20/15-2 in the right eye and 20/15 in the aware

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multiple new scotomata were plotted on a tangent screen with a 2/1,000 left eye,

white test target. No biomicroscopic ab¬ normalities of the anterior segment were seen. The ophthalmoscopic examination showed a new crop of creamy-yellow subretinal lesions in previously uninvolved areas of the posterior pole of each eye. However, the original lesions had involuted into a patchwork of irregular coarse pig¬ ment clumping and depigmented zones

(Fig 5).

A further medical investigation included the following studies, which were normal or negative: complete blood count, erythrocyte sedimentation rate, serum creatinine, blood urea nitrogen, electrolytes, alkaline phosphatase, lactic dehydrogenase, glutamic-oxaloacetic transaminase, uric acid, bilirubin, protein electrophoresis, antinuclear factor, latex rheumatoid factor, and lupus erythematosus preparation. Administration of prednisone (100 mg/day) was started again, and prophy¬ lactic isoniazid (300 mg/day) was added. Oral contraceptives were discontinued, and an intrauterine device implanted. During the succeeding three weeks, the lesions became quiescent in the same fash¬ ion as the original clusters, with progres¬ sive depigmentation and pigment clump¬ ing. The dosage of corticosteroids was tapered more slowly and terminated by mid-October 1971. The placoid lesions

cleared, leaving a patchy clumped pigment disturbance in the posterior pole of each eye. Since that time, no funduscopic changes have occurred (Fig 6). When last seen in June 1973, her vision was recorded

phase shows early staining of macular le¬

Fig 4.—Forty-five minutes after fluorescein injection, bright fluorescent staining of macular lesions remains. Angiographie behavior of multifocal lesions in left eye was similar.

Fig 5.—New crop of creamy-yellow multifocal subretinal lesions appeared in previously uninvolved areas of each eye on July 22, 1971. Initial lesions have resolved, leaving mottled patches of clumped pigment.

Fig 6.—Twenty months after initial episode, irregular scars of patchy pigment clumping and depigmentation remain in posterior pole of right eye. Left eye showed similar evolution.

Fig 3.—Late

venous

sions.

as

20/15-2 and

only complaint phopsia in each

Jaeger was

0 in each eye. Her slight metamor-

of

eye.

COMMENT

Acute posterior multifocal placoid pigment epitheliopathy appears to be a specific clinical entity. In spite of the similarity in features reported by Gass1 and subsequent authors,59 it is likely that the disorder is not new but

has been classified previously as a multifocal or disseminated choroiditis. Thirty-one patients have been sufficiently described in the American literature, in addition to our patient, to allow some generalizations about the clinical features, the clinical course, and the prognosis of the dis¬ order. Of the 31 patients, 29 ranged from 11 years old to 38 years old, one being 8 years and one being 57 years.

The average age at presentation is about 23 years, the age of our patient. Seventeen were male, and 14 were fe¬ male, thus discarding the impression from the early literature that the dis¬ order was limited to females. Of the 31 previously described pa¬ tients, 27 were white, two were black, and the race was not specified in two patients. These data may reflect the bias of populations in the reporting

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clinics rather than a racial predilec¬ tion of the disease. Twenty-nine of the previously de¬ scribed patients had both eyes in¬ volved; two patients had only one eye afflicted. Anterior segment involve¬ ment, either as iritis or episcleritis, has been reported in six patients, and vitreous involvement, as was tran¬ siently noted during the initial epi¬ sode in our patient, has been seen in only two others.

Clinically, patients develop

a

rapid

loss of central vision. Ophthalmoscopy shows multiple yellow-white placoid lesions at the level of the retinal pig¬

epithelium or choriocapillaris. commonly, they cover the poste¬ rior pole but may extend anteriorly to the equator. Rarely, retinal edema or small retinal hemorrhages have been ment

Most

observed.8

Usually, in two to five weeks, the placoid lesions spontaneously resolve leaving scattered areas of depigmen¬ tation and pigment clumping. Sub¬ stantial visual improvement contin¬ ues for several weeks or months, even after apparent ophthalmoscopic reso¬

lution of the acute lesions. Over 80% of reported affected eyes have shown an ultimate visual acuity equal to or better than 20/40. No important contributions from the history or general physical exami¬ nation have been identified among the previously reported 31 patients. Four of them had antecedent respira¬ tory and five had antecedent gas¬ trointestinal illnesses"-8 resembling influenza. The disease has also been described in a patient with erythema nodosum following penicillin therapy for streptococcal pharyngitis0 and in a young girl with systemic mycobacterial infection." Oral contraceptives

"hormones" were used by only two of the 14 female patients who have been described in detail1-8 and, there¬ fore, do not seem to relate to the dis¬ or

ease.

in the course of the disease, fluorescein angiography shows welldefined areas of hypofluorescent screening of the choroidal blush dur¬ ing the initial transit of dye. The spots are discrete and constant in size until the late venous phase when they become hyperfluorescent compared to the adjacent pigment epithelium. These spots are distinctly different in size and course from the geographic patches of choroidal nonfilling seen early in the normal angiogram. Gass12 thought that the fluorescein screening was due to the cloudy swell¬ ing of locally diseased retinal pig¬ ment epithelium and argued that this abnormal pigment epithelium later absorbed fluorescein, implying dam¬ age to the integrity of the metabolic barrier at Buch's membrane; van Buskirk et al" showed slow, irregular fill¬ ing of the choroidal capillaries and concluded that the changes could be explained by a focal choroidal vasculopathy rather than a primary pig¬

Early

ment

epitheliopathy.

The ophthalmoscopic, biomicroscopic, and angiographie appearance of each of the two crops of lesions in our patient adheres closely to the descrip¬ tion by Gass.1 Angiographie changes after resolution of the acute lesions shows damage to the retinal pigment epithelium and not to the choroid or retina. About one third of the reported pa¬ tients have been treated with cortico¬ steroids by various routes of adminis¬ tration. One of the cases presented by Ryan and Maumenee" (case 7) noted -

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prompt improvement after starting

systemic steroid therapy and subse¬ quent decrease in acuity when steroid therapy was tapered, obligating its

reinstitution. The authors do not state whether new ophthalmoscopic lesions were responsible for the change in visual acuity. There is no difference in the course of the disease, the duration of visual loss, and the ex¬ tent of visual improvement among the reported cases treated with steroids. Nor do steroids appear to have substantially affected the evolu¬ tion of the disease in our patient. The role of steroids in effecting resolution of the placoid lesions is, therefore, un¬ likely. The selection of specific ther¬ apy may wait until the demonstration of the pathogenetic mechanism of the disorder. This study was supported in part by a grant from Research to Prevent Blindness, Inc., New York.

References 1. Gass JDM: Acute posterior multifocal placoid pigment epitheliopathy. Arch Ophthalmol 80:177-185, 1968. 2. Gass JDM: Stereoscopic Atlas of Macular Diseases. St. Louis, CV Mosby, 1970, p 128. 3. Schlaegel PF Jr: Essentials of Uveitis. Boston, Little Brown & Co, 1969, p 103. 4. Maumenee AE: Clinical entities in uveitis: An approach to the study of intraocular inflammation. Am J Ophthalmol 69:1-27, 1970. 5. van Buskirk EM, Lessel S, Friedman E: Pigment epitheliopathy and erythema nodosum. Arch Ophthalmol 85:369-372, 1971. 6. Ryan SJ, Maumenee AE: Acute posterior multifocal placoid pigment epitheliopathy. Am J Ophthalmol 74:1066-1074, 1972. 7. Fitzpatrick RJ, Robertson DM: Acute posterior multifocal placoid pigment epitheliopathy. Arch Ophthalmol 89:373-376, 1973. 8. Annesley WH, Tomer TL, Shields JA: Multifocal placoid pigment epitheliopathy. Am J Ophthalmol 76:511-518, 1973. 9. Brown M, Eberdt A, Ladas G: Pigment epitheliopathy in a patient with mycobacterial infection. J Pediatr Ophthalmol 10:278-281, 1973.

Acute posterior multifocal placoid pigment epitheliopathy. A recurrence.

Acute posterior multifocal placoid pigment epitheliopathy recurred in a 23-year-old white woman. Forty-two days after the onset of symptoms and follow...
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