Epidermolysis bullosa

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5. Musculokeletal deformities

The musculoskeletal system deformities are a frequent problem in people with severe types of EB and, apart from causing a functional limitation, they are also the origin of many psychological problems. 

 

The most important visible musculoskeletal complication of EB is pseudodactyly, commonly known as the “claw or mitten hand/foot”. This injury is especially frequent in the generalized dystrophic type, and it is produced due to the repetitive presence of blisters between the fingers or toes which favours the synechiae (merging) of the interdigital areas of one or more fingers. In more severe forms all the extremities’ fingers/toes can be united limiting the hand movement and its functioning in big measure. If the pseudodactyly is not corrected early through surgery, the muscles left unused can end up atrophying.

sindactilia

 

Another particularly frequent musculoskeletal problem are the contractures. Ordered by frequency, they happen in the fingers and toes, legs, axillary and inguinal areas and the base of the neck. These contractures can appear due to not using the muscular groups of the contracture area (due to a malformation or due to being confined in a bed or wheelchair) or due to the accumulation of granulation tissue (injury reparation tissue) because it does not finish healing. 
These deformities are manifested in the early life stages. It is very important to be conscious of this and start as soon as possible with the non-surgical measures, like physiotherapy for not losing strength and mobility. To reduce the risk of severe malformations splints and bandages can be used. 
Last modified
25 March 2025