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What are Zebra Bodies ?

 Dr Sampurna Roy MD   

 

                                                                                                                      

 

                          

 

 A)

 

B) 

A and B = Electron micrograph showing concentric lamination of inclusion bodies.

 

Answer:  Fabry disease (Syn: Fabry's disease ; Anderson-Fabry Disease)

The first descriptions of Fabry disease were made in 1898 by two physicians.William Anderson and Johannes Fabry described patients with 'angiokeratoma corporis diffusum', the red–purple maculopapular skin lesions that are now recognized as a characteristic feature of the disorder.    Angiokeratoma

Fabry disease, is also referred to as Anderson–Fabry disease in recognition of the original descriptions made in 1898 by Anderson and Fabry. History of lysosomal storage diseases: an overview.

Fabry disease is a rare X-linked recessive  lysosomal storage  disorder. Mutations of the GLA  gene result in deficiency of the lysosomal enzyme α-galactosidase A.

There is accumulation of glycosphingolipids, particularly globotriaosylceramide (GL3) in various organs. Example: Heart, kidneys, liver, skin, central nervous system, and lungs.

Progressive accumulation of substrate in various organs is associated with renal failure, cardiovascular dysfunction, neuropathy, stroke and dermatological manifestations in the form of angiokeratomas.

Electron microscopy shows diagnostic intracytoplasmic inclusions having a lamellar structure - These are ZEBRA BODIES.

Interesting articles:

Paediatric Fabry disease.

Fabry disease, respiratory symptoms, and airway limitation - a systematic review.

Related post: Pathology of Angiokeratoma

 

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)

 

 


 

 

 

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