Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is an acquired inflammatory

uveitis Uveitis () is inflammation of the uvea, the pigmented layer of the eye between the inner retina and the outer fibrous layer composed of the sclera and cornea. The uvea consists of the middle layer of pigmented vascular structures of the eye and ...

that belongs to the heterogenous group of

white dot syndromes White dot syndromes are inflammatory diseases characterized by the presence of white dots on the fundus (eye), fundus, the interior surface of the eye.macular A skin condition, also known as cutaneous condition, is any medical condition that affects the integumentary system—the organ system that encloses the body and includes skin, nails, and related muscle and glands. The major function of this ...

area of the retina. Early in the course of the disease, the lesions cause acute and marked

vision loss Visual impairment, also known as vision impairment, is a medical definition primarily measured based on an individual's better eye visual acuity; in the absence of treatment such as correctable eyewear, assistive devices, and medical treatment� ...

(if it interferes with the optic nerve) that ranges from mild to severe but is usually transient in nature. APMPPE is classified as an inflammatory disorder that is usually bilateral and acute in onset but self-limiting. The lesions leave behind some pigmentation, but

visual acuity Visual acuity (VA) commonly refers to the clarity of vision, but technically rates an examinee's ability to recognize small details with precision. Visual acuity is dependent on optical and neural factors, i.e. (1) the sharpness of the retinal ...

eventually improves even without any treatment (providing scarring doesn't interfere with the optic nerve). It occurs equally between men and women with a male to female ratio of 1.2:1. Mean onset age is 27, but has been seen in people aged 16 to 40. It is known to occur after or concurrently with a systemic infection (but not always), showing that it is related generally to an altered

immune system The immune system is a network of biological processes that protects an organism from diseases. It detects and responds to a wide variety of pathogens, from viruses to parasitic worms, as well as cancer cells and objects such as wood splinte ...

. Recurrent episodes can happen, but are extremely rare.

Signs and symptoms

The onset of ocular symptoms are usually preceded by episode of viral or flu-like symptoms such as fever, cough or sore throat (however this is not always the case). Patients can typically present erythema nodosum, livido reticularus, bilateral uveitis, and sudden onset of marked visual loss associated with the appearance of multiple lesions in the retina. These lesions may be colored from grey-white to cream-shaded yellow. Other symptoms include scotomata and

photopsia Photopsia is the presence of perceived flashes of light in the Visual field, field of vision. It is most commonly associated with: * posterior vitreous detachment * migraine aura (ocular migraine / retinal migraine) * acephalgic migraine, migrai ...

. In weeks to a month times the lesions begin to clear and disappear (with prednisone) leaving behind areas of retinal pigment epithelial atrophy and diffuse fine pigmentation (scarring). Rarely

choroidal neovascularization Choroidal neovascularization (CNV) is the creation of new blood vessels in the choroid layer of the eye. Choroidal neovascularization is a common cause of neovascular degenerative maculopathy (i.e. 'wet' macular degeneration) commonly exacerbated ...

occur as a late onset complication.

Cause

Since The cause of the inflammation remains unknown, with various theories of it occurring as an autoimmune response to a mild infection, or the possibility of it being viral because of the preceding flu-like illness that generally accompanies it. It is associated an increased incidence of both

HLA-B7 HLA-B7 (B7) is an HLA- B serotype. The serotype identifies the more common HLA-B*07 gene products. (For terminology help see: HLA-serotype tutorial) B7, previously HL-A7, was one of the first 'HL-A' antigens recognized, largely because of the f ...

and

HLA-DR2 HLA-DR2 (DR2) of the HLA- DR serotype system, is a broad antigen serotype that is now preferentially covered by HLA-DR15 and HLA-DR16 serotype group. This serotype primarily recognizes gene products of the HLA-DRB1*15 and HLA-DRB1*16 allele gro ...

. The underlying etiology of APMPPE continues to cause debate. The term 'Pigment Epitheliopathy' was chosen by Gass to reflect what he thought was the tissue most significantly affected. Van Buskirk et al., and Deutman et al. proposed choriocapillaris ischemia as the more likely primary etiology. Indocyanine green angiography (ICGA), and OCT angiography (OCTA) studies have provided support for choriocapillaris involvement. However, a novel hypothesis was proposed implicating a direct neurotropic infection as a possible underlying cause given the dynamic changes observed along the neuronal pathway of the retina 1. Steptoe PJ, Pearce I, Beare NAV, Sreekantam S, Mohammed BR, Barry R, Steeples LR, Denniston AK. Proposing a Neurotropic Etiology for Acute Posterior Multifocal Placoid Pigment Epitheliopathy and Relentless Placoid Choroidopathy. Investigative Ophthalmology & Visual Science (2021) 62:3447–3447. doi:10.3389/fopht.2021.802962 https://www.frontiersin.org/articles/10.3389/fopht.2021.802962/full

Diagnosis

Diagnosis is usually made on clinical appearance alone on

fundoscopy Ophthalmoscopy, also called funduscopy, is a test that allows a health professional to see inside the fundus of the eye and other structures using an ophthalmoscope (or funduscope). It is done as part of an eye examination and may be done as part ...

and/or retinal imaging. Supplementary tests such as Optical coherence tomography(OCT) and fundus fluorescein angiography/Indocyanine angiography together with OCT-Angiography are commonly performed to help aid diagnosis and monitoring.

Fundus photography Fundus photography involves photographing the rear of an eye, also known as the fundus. Specialized fundus cameras consisting of an intricate microscope attached to a flash enabled camera are used in fundus photography. The main structures that ...

is useful to document the appearance of APMPPE lesions.

Management

The original description categorized APMPPE as a self-limiting condition with a good prognosis, but the disease can be recurrent and result in significant visual loss. Owing to the self-limiting nature of the disease, treatment is generally not required. In cases where lesions appear to be interfering with the optic nerve, methyl

prednisone Prednisone is a glucocorticoid medication mostly used to immunosuppressive drug, suppress the immune system and decrease inflammation in conditions such as asthma, COPD, and rheumatologic diseases. It is also used to treat high blood calcium ...

is prescribed, but in recurrent disease an

antimetabolite An antimetabolite is a chemical that inhibits the use of a metabolite, which is another chemical that is part of normal metabolism. Such substances are often similar in structure to the metabolite that they interfere with, such as the antifolate ...

agent may be indicated.

Prognosis

Vision improves in almost all cases. In rare cases, a patient may suffer permanent visual loss associated with lesions on their optic nerve. Rarely, coexisting

vasculitis Vasculitis is a group of disorders that destroy blood vessels by inflammation. Both arteries and veins are affected. Lymphangitis (inflammation of lymphatic vessels) is sometimes considered a type of vasculitis. Vasculitis is primarily caused b ...

may cause neurological complications. These occurrences can start with mild headaches that steadily worsen in pain and onset, and can include attacks of

dysesthesia Dysesthesia is an unpleasant, abnormal sense of touch. Its etymology comes from the Greek word "dys," meaning "bad," and "aesthesis," which means "sensation" (abnormal sensation). It often presents as painfovea Fovea () (Latin for "pit"; plural foveae ) is a term in anatomy. It refers to a pit or depression in a structure. Human anatomy *Fovea centralis of the retina * Fovea buccalis or Dimple * Fovea of the femoral head * Trochlear fovea of the fr ...

References

External links

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