Herpes zoster ophthalmicus (HZO), also known as ophthalmic zoster, is

shingles Shingles, also known as zoster or herpes zoster, is a viral disease characterized by a painful skin rash with blisters in a localized area. Typically the rash occurs in a single, wide mark either on the left or right side of the body or face. ...

involving the eye or the surrounding area. Common signs include a

rash A rash is a change of the human skin which affects its color, appearance, or texture. A rash may be localized in one part of the body, or affect all the skin. Rashes may cause the skin to change color, itch, become warm, bumpy, chapped, dry, cr ...

of the forehead with swelling of the eyelid. There may also be eye pain and redness, inflammation of the conjunctiva,

cornea The cornea is the transparent front part of the eye that covers the iris, pupil, and anterior chamber. Along with the anterior chamber and lens, the cornea refracts light, accounting for approximately two-thirds of the eye's total optical power ...

uvea The uvea (; Lat. ''uva'', "grape"), also called the ''uveal layer'', ''uveal coat'', ''uveal tract'', ''vascular tunic'' or ''vascular layer'' is the pigmented middle of the three concentric layers that make up an eye. History and etymolog ...

, and sensitivity to light. Fever and tingling of the skin and

allodynia Allodynia is a condition in which pain is caused by a stimulus that does not normally elicit pain. For example, bad sunburn can cause temporary allodynia, and touching sunburned skin, or running cold or warm water over it, can be very painful. It i ...

near the eye may precede the rash. Complications may include

visual impairment 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� ...

, increased pressure within the eye,

chronic pain Chronic pain is classified as pain that lasts longer than three to six months. In medicine, the distinction between Acute (medicine), acute and Chronic condition, chronic pain is sometimes determined by the amount of time since onset. Two commonly ...

, and

stroke A stroke is a medical condition in which poor blood flow to the brain causes cell death. There are two main types of stroke: ischemic, due to lack of blood flow, and hemorrhagic, due to bleeding. Both cause parts of the brain to stop functionin ...

. The underlying mechanism involves a reactivation of the

latent Latency or latent may refer to: Science and technology * Latent heat, energy released or absorbed, by a body or a thermodynamic system, during a constant-temperature process * Latent variable, a variable that is not directly observed but inferred ...

varicella zoster virus Varicella-zoster virus (VZV), also known as human herpesvirus 3 (HHV-3, HHV3) or ''Human alphaherpesvirus 3'' (taxonomically), is one of nine known herpes viruses that can infect humans. It causes chickenpox (varicella) commonly affecting chil ...

(VZV) within the

trigeminal ganglion A trigeminal ganglion (or Gasserian ganglion, or semilunar ganglion, or Gasser's ganglion) is the sensory ganglion at the base of each of the two trigeminal nerves (CN V), occupying a cavity ( Meckel's cave) in the dura mater, covering the trige ...

supplying the

ophthalmic nerve The ophthalmic nerve (V1) is a sensory nerve of the face. It is one of three divisions of the trigeminal nerve (CN V). It has three branches that provide sensory innervation to the eye, the skin of the upper face, and the skin of the anterior sca ...

(the first division of the

trigeminal nerve In neuroanatomy, the trigeminal nerve ( lit. ''triplet'' nerve), also known as the fifth cranial nerve, cranial nerve V, or simply CN V, is a cranial nerve responsible for sensation in the face and motor functions such as biting and chewin ...

). Diagnosis is generally based on signs and symptoms. Alternatively, fluid collected from the rash may be analyzed for VZV DNA using

real-time PCR A real-time polymerase chain reaction (real-time PCR, or qPCR) is a laboratory technique of molecular biology based on the polymerase chain reaction (PCR). It monitors the amplification of a targeted DNA molecule during the PCR (i.e., in real ...

. This test is rapid, easy to perform, and is highly sensitive and specific method for diagnosing this condition. Treatment is generally with antiviral pills such as

acyclovir Aciclovir (ACV), also known as acyclovir, is an antiviral medication. It is primarily used for the treatment of herpes simplex virus infections, chickenpox, and shingles. Other uses include prevention of cytomegalovirus infections following tran ...

. Steroid eye drops and drops to dilate the pupil may also be used. The

herpes zoster vaccine A zoster vaccine is a vaccine that reduces the incidence of herpes zoster (shingles), a disease caused by reactivation of the varicella zoster virus, which is also responsible for chickenpox. Shingles provokes a painful rash with blisters, and ...

is recommended for prevention in those over the age of 50. HZO is the second most common manifestation of shingles, the first being involvement of skin of the thorax. Shingles affects up to one half million people in the United States per year, of which 10% to 25% is HZO.

Signs and symptoms

Skin

Trigeminal herpes with uveitis and keratitis

* Viral

prodrome In medicine, a prodrome is an early sign or symptom (or set of signs and symptoms) that often indicates the onset of a disease before more diagnostically specific signs and symptoms develop. It is derived from the Greek word ''prodromos'', meaning ...

Preherpetic neuralgia Preherpetic neuralgia is a form of nerve pain ( neuralgia) specifically associated with a Shingles (herpes zoster) viral infection. This nerve pain often precedes visible indications of a Shingles infection and consequently can be a key early indica ...

* Rash, transitioning from

papule A papule is a small, well-defined bump in the skin. It may have a rounded, pointed or flat top, and may have a dip. It can appear with a stalk, be thread-like or look warty. It can be soft or firm and its surface may be rough or smooth. Some h ...

s to

vesicle Vesicle may refer to: ; In cellular biology or chemistry * Vesicle (biology and chemistry), a supramolecular assembly of lipid molecules, like a cell membrane * Synaptic vesicle ; In human embryology * Vesicle (embryology), bulge-like features o ...

s to

pustule 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 sy ...

s to scabs. * Hutchinson's sign:

cutaneous Skin is the layer of usually soft, flexible outer tissue covering the body of a vertebrate animal, with three main functions: protection, regulation, and sensation. Other animal coverings, such as the arthropod exoskeleton, have different de ...

involvement of the tip of the nose, indicating

nasociliary nerve The nasociliary nerve is a branch of the ophthalmic nerve, itself a branch of the trigeminal nerve (CN V). It is intermediate in size between the other two branches of the ophthalmic nerve, the frontal nerve and lacrimal nerve. Structure The naso ...

involvement. While a positive Hutchinson's sign increases the likelihood of ocular complications associated with HZO, it's absence does not rule out ophthalmic involvement. * Disseminated distribution in individuals with

immunodeficiency Immunodeficiency, also known as immunocompromisation, is a state in which the immune system's ability to fight infectious diseases and cancer is compromised or entirely absent. Most cases are acquired ("secondary") due to extrinsic factors that a ...

Cornea

* Epithelial:

punctate epithelial erosions Punctate epithelial erosions are a pathology affecting the cornea. Signs and symptoms It is a characterized by a breakdown or damage of the epithelium of the cornea in a pinpoint pattern, which can be seen with examination with a slit-lamp. ...

and pseudodendrites: often have anterior stromal infiltrates. Onset 2 to 3 days after the onset of the rash, resolving within 2–3 weeks. Common. * Stromal: **

Nummular keratitis Nummular keratitis is a feature of viral keratoconjunctivitis. It is a common feature of adenoviral keratoconjunctivitis (an ocular adenovirus infection), as well as approximately 1/3rd of cases of Herpes Zoster Ophthalmicus infections. It repr ...

: have anterior stromal granular deposits. Occurs within 10 days of onset of rash. Uncommon ** Necrotising interstitial keratitis: Characterised by stromal infiltrates, corneal thinning and possibly perforation. Occurs between 3 months and several years after the onset of rash. Rare. * Disciform Keratitis (''Disciform Endotheliitis''): a disc of corneal oedema, folds in

Descemet's membrane Descemet's membrane ( or the Descemet membrane) is the basement membrane that lies between the corneal proper substance, also called stroma, and the endothelial layer of the cornea. It is composed of different kinds of collagen (Type IV and VIII) ...

, mild inflammation evident within the

anterior chamber The anterior chamber ( AC) is the aqueous humor-filled space inside the eye between the iris and the cornea's innermost surface, the endothelium. Hyphema, anterior uveitis and glaucoma are three main pathologies in this area. In hyphema, blood f ...

and fine keratic precipitates. Chronic. Occurs between 3 months and several years after the onset of the rash. Uncommon. *

Neurotrophic Neurotrophic factors (NTFs) are a family of biomolecules – nearly all of which are peptides or small proteins – that support the growth, survival, and differentiation of both developing and mature neurons. Most NTFs exert their tro ...

: corneal nerve damage causes a persistent epithelial defect, thinning and even perforation. The cornea becomes susceptible to bacterial and fungal

keratitis Keratitis is a condition in which the eye's cornea, the clear dome on the front surface of the eye, becomes inflamed. The condition is often marked by moderate to intense pain and usually involves any of the following symptoms: pain, impaired e ...

. Chronic. Late onset. Uncommon. * Mucous plaques: linear grey elevations loosely adherent to the underlying diseased epithelium/stroma. Chronic. Onset between 3 months and several years after the onset of the rash.

Uveal

Anterior uveitis develops in 40–50% of people with HZO within 2 weeks of the onset of the skin rashes. Typical HZO keratitis at least mild

iritis 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 ...

, especially if Hutchinson's sign is positive for the presence of

s upon the tip of the nose. Features: This non-

granulomatous A granuloma is an aggregation of macrophages that forms in response to chronic inflammation. This occurs when the immune system attempts to isolate foreign substances that it is otherwise unable to eliminate. Such substances include infectiou ...

iridocyclitis 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 i ...

is associated with: * Small keratic precipitates * Mild aqueous flare * Occasionally haemorrhagic

hypopyon Hypopyon is a medical condition involving inflammatory cells in the anterior chamber of the eye. It is an exudate rich in white blood cells, seen in the anterior chamber, usually accompanied by redness of the conjunctiva and the underlying epis ...

HZO

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 ...

is associated with complications such as iris atrophy and

secondary glaucoma Secondary glaucoma is a collection of progressive optic nerve disorders associated with a rise in intraocular pressure (IOP) which results in the loss of vision. In clinical settings, it is defined as the occurrence of IOP above 21 mmHg requiring th ...

are not uncommon. Complicated

cataract A cataract is a cloudy area in the lens of the eye that leads to a decrease in vision. Cataracts often develop slowly and can affect one or both eyes. Symptoms may include faded colors, blurry or double vision, halos around light, trouble w ...

may develop in the late stages of the disease.

Anatomy

HZO is due to reactivation of VZV within the trigeminal ganglion. The trigeminal ganglion give rise to the three divisions of cranial nerve V (CN V), namely the ophthalmic nerve, the maxillary nerve, and the mandibular nerve. VZV reactivation in trigeminal ganglion predominantly affects the ophthalmic nerve, for reasons not clearly known. The ophthalmic nerve gives rise to three branches: the supraorbital nerve, the supratrochlear nerve, and the nasociliary nerve. Any combination of these nerves can be affected in HZO, although the most feared complications occur with nasociliary nerve involvement, due to its innervation of the eye. The supraorbital and supratrochlear nerves mainly innervate the skin of the forehead. The

frontal nerve The frontal nerve is the largest branch of the ophthalmic nerve (V1), itself a branch of the trigeminal nerve (CN V). It supplies sensation to the skin of the forehead, the mucosa of the frontal sinus, and the skin of the upper eyelid. It may be ...

is more commonly affected than the

lacrimal nerve The lacrimal nerve is the smallest branch of the ophthalmic nerve (V1), itself a branch of the trigeminal nerve (CN V). The other branches of the ophthalmic nerve are the frontal nerve and nasociliary nerve. Structure The lacrimal nerve branche ...

Treatment

Treatment is usually with antivirals such as

valacyclovir Valaciclovir, also spelled valacyclovir, is an antiviral medication used to treat outbreaks of herpes simplex or herpes zoster (shingles). It is also used to prevent cytomegalovirus following a kidney transplant in high risk cases. It is taken ...

, or famcyclovir by mouth. There is uncertainty as to the difference in effect between these three antivirals. Antiviral eye drops have not been found to be useful. These medications work best if started within 3 days of the start of the rash. Cycloplegics prevent synechiae from forming.

References

External links

{{Varicella zoster Varicella zoster virus-associated diseases Ophthalmology Wikipedia medicine articles ready to translate