Griscelli Syndrome & Skin Pigmentation


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Griscelli syndrome is a disorder of partial albinism with deficiency of the immune system. It causes a decrease in the amount of pigmentation in the skin and the hair.

What does Griscelli syndrome skin pigmentation look like?

The main symptom that people notice in sufferers of Griscelli syndrome is silver hair. There are several skin conditions associated with Griscelli syndrome such as skin lesions and partial albinism. Skin in people with Griscelli syndrome is usually very pale but not as severe as in those with complete albinism.

What are the causes of Griscelli syndrome skin pigmentation?

Griscelli syndrome is a genetic disorder which can be inherited. It causes there to be large clumps of pigment in the hair.

There are three genes which can be mutated in order to cause Griscelli syndrome. The exact symptoms of Griscelli syndrome depend on which gene is mutated.

Who is likely to get Griscelli syndrome?

Griscelli syndrome is a very rare disease and there have been very few cases reported in the United States or the United Kingdom. It is most commonly found in Turkish and Mediterranean countries as well as in India. Both men and women can be affected by this condition.

Is Griscelli syndrome dangerous?

Griscelli syndrome causes people suffering from it to die unless they receive a bone marrow transplant. The average age at which people die from the condition is 5 years old and it usually develops in people aged between 4 months and 4 years old.

How is Griscelli syndrome treated?

The only treatment for Griscelli syndrome is a bone marrow transplant, in which stem cells are transplanted. This extends survival but does not cure the neurological problems linked with Griscelli syndrome.


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