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Alezzandrini syndrome is a very rare syndrome characterized by a unilateral degenerative
retinitis Retinitis is inflammation of the retina in the eye, which can permanently damage the retina and lead to blindness. The retina is the eye's "sensing" tissue. Retinitis may be caused by a number of different infectious agents. Its most common form, ...
, followed after several months by ipsilateral
vitiligo Vitiligo is a disorder that causes the skin to lose its color. Specific causes are unknown but studies suggest a link to immune system changes. Signs and symptoms The only sign of vitiligo is the presence of pale patchy areas of depigmen ...
on the face and ipsilateral
poliosis Poliosis (also called poliosis circumscripta), is the decrease or absence of melanin (or colour) in head hair, eyebrows, eyelashes or any other hairy area. It is popularly known as white forelock when it affects hair directly above the forehead. ...
.
Deafness Deafness has varying definitions in cultural and medical contexts. In medical contexts, the meaning of deafness is hearing loss that precludes a person from understanding spoken language, an audiological condition. In this context it is written ...
may also be present.


Signs and symptoms

Initially, there is a progressive loss of visual acuity, mostly in one eye. Infected areas experience pigmentation loss in the skin and hair. Unilateral tapetoretinal degeneration accompanied by the ipsilateral appearance of facial
vitiligo Vitiligo is a disorder that causes the skin to lose its color. Specific causes are unknown but studies suggest a link to immune system changes. Signs and symptoms The only sign of vitiligo is the presence of pale patchy areas of depigmen ...
and
poliosis Poliosis (also called poliosis circumscripta), is the decrease or absence of melanin (or colour) in head hair, eyebrows, eyelashes or any other hairy area. It is popularly known as white forelock when it affects hair directly above the forehead. ...
is the hallmark of Alezzandrini syndrome.


Causes

It is unknown what causes Alezzandrini syndrome.


Diagnosis

The clinical presentation is the basis for the diagnosis.


Treatment

Psoralen plus ultraviolet A PUVA (psoralen and UVA) is an ultraviolet light therapy treatment for skin diseases: eczema, psoriasis, graft-versus-host disease, vitiligo, mycosis fungoides, large plaque parapsoriasis, and cutaneous T-cell lymphoma, using the sensitizing effec ...
(PUVA) treatment is an option for cases with extensive depigmentation.
Topical steroid Topical steroids are the topical forms of corticosteroids. Topical steroids are the most commonly prescribed topical medications for the treatment of rash, eczema, and dermatitis. Topical steroids have anti-inflammatory properties and are classifie ...
s may be used to treat
vitiligo Vitiligo is a disorder that causes the skin to lose its color. Specific causes are unknown but studies suggest a link to immune system changes. Signs and symptoms The only sign of vitiligo is the presence of pale patchy areas of depigmen ...
in specific areas. People who have
vitiligo Vitiligo is a disorder that causes the skin to lose its color. Specific causes are unknown but studies suggest a link to immune system changes. Signs and symptoms The only sign of vitiligo is the presence of pale patchy areas of depigmen ...
should wear sunscreen to avoid getting sunburned and developing
skin cancer Skin cancers are cancers that arise from the skin. They are due to the development of abnormal cells that have the ability to invade or spread to other parts of the body. There are three main types of skin cancers: basal-cell skin cancer (BCC) ...
later on.


See also

*
List of cutaneous conditions Many skin conditions affect the human integumentary system—the organ system covering the entire surface of the body and composed of skin, hair, nails, and related muscle and glands. The major function of this system is as a barrier against t ...
*
Skin lesion 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 s ...


References


External links


DermNet
{{Medical resources , ICD11 = {{ICD11, ED63.Y , ICD10 = {{ICD10, L81.8 , ICD10CM = , ICD9 = , ICDO = , OMIM = , MeshID = , DiseasesDB = 34138 , SNOMED CT = 403275004 , Curlie = , MedlinePlus = , eMedicineSubj = article , eMedicineTopic = 1117255 , PatientUK = , NCI = , GeneReviewsNBK = , GeneReviewsName = , NORD = , GARDNum = 13023 , GARDName = Alezzandrini syndrome , RP = , AO = , WO = , OrthoInfo = , Orphanet = , Scholia = Q4721578 , OB = Disturbances of human pigmentation Rare syndromes Syndromes affecting the retina Syndromes affecting hearing Syndromes affecting the skin