Alezzandrini syndrome

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Alezzandrini syndrome
Synonyms Alezzandrini syndrome
Pronounce N/A
Specialty N/A
Symptoms Unilateral vitiligo, poliosis, retinal detachment, hearing loss
Complications Vision loss, hearing impairment
Onset Typically in adulthood
Duration Chronic
Types N/A
Causes Idiopathic
Risks Unknown
Diagnosis Clinical diagnosis
Differential diagnosis Vogt-Koyanagi-Harada disease, Waardenburg syndrome
Prevention N/A
Treatment Symptomatic treatment
Medication Corticosteroids, immunosuppressants
Prognosis Variable, depends on severity of symptoms
Frequency Rare
Deaths N/A


A rare disorder affecting the skin, eyes, and ears


Template:Medical condition (new) Alezzandrini syndrome is a rare disorder characterized by a combination of skin, eye, and ear abnormalities. It is considered a type of autoimmune disorder, where the body's immune system mistakenly attacks its own tissues.

Presentation[edit | edit source]

Alezzandrini syndrome typically presents with a triad of symptoms:

Ocular Symptoms[edit | edit source]

The most prominent feature of Alezzandrini syndrome is the involvement of the eye. Patients may experience unilateral retinal detachment, leading to vision loss in one eye. Other ocular manifestations can include uveitis, vitiligo of the retina, and optic atrophy.

Cutaneous Symptoms[edit | edit source]

The skin manifestations of Alezzandrini syndrome often include vitiligo, which is the loss of pigmentation in patches of skin. This depigmentation is usually unilateral and may be associated with poliosis, a condition where hair in the affected area turns white.

Auditory Symptoms[edit | edit source]

Hearing loss is another significant feature of Alezzandrini syndrome. Patients may experience sensorineural hearing loss, which can be progressive and may affect one or both ears.

Pathophysiology[edit | edit source]

The exact cause of Alezzandrini syndrome is not well understood, but it is believed to be an autoimmune process. The immune system attacks the melanocytes, which are the cells responsible for producing melanin, the pigment that gives color to the skin, hair, and eyes. This autoimmune attack leads to the characteristic symptoms of the syndrome.

Diagnosis[edit | edit source]

Diagnosis of Alezzandrini syndrome is primarily clinical, based on the presence of the characteristic triad of symptoms. Ophthalmologic examination, audiometry, and dermatologic evaluation are essential for confirming the diagnosis. Imaging studies such as MRI may be used to assess the extent of involvement in the eyes and ears.

Management[edit | edit source]

There is no cure for Alezzandrini syndrome, and treatment is primarily symptomatic. Management may include:

Prognosis[edit | edit source]

The prognosis for individuals with Alezzandrini syndrome varies. While some patients may experience a stable course, others may have progressive symptoms leading to significant visual and auditory impairment.

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Contributors: Prab R. Tumpati, MD