What is Kindler’s Syndrome?

What is Kindler’s Syndrome?

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By Dr. Rasya Dixit, Dermatology

Kindler’s Syndrome is a rare type of epidermolysis bullosa (EB). EB is a group of inherited diseases that is characterised by skin fragility, blistering of skin and discoloration. This syndrome is quite common in early infancy. Kindler’s syndrome shows mixed patches of blistering on the skin with multiple levels on the epidermis and beneath the epidermis. It is an autosomal recessive inherited disorder.

Symptoms behind this disease-

There are lots of symptoms shown by this disease.Most common of them are:

  • Abnormal blistering of skin.
  • Widespread skin breakdown.
  • Thickening and hardening of skin on the palms of hands and soles of feet.
  • Photosensitivity: Includes increased sensitivity to light.
  • Patchy discoloration of skin.
  • Feeding difficulties in infancy.
  • Any traumatic incident related to hands and feet.
  • Development of dental abnormalities.
  • Skin ulcer: Burns are formed on the skin from mild ones to blisters.
  • Gingivitis.
  • Loss of primary teeth in early ages.
  • Periodontitis.
  • Abnormality of the gastric mucosa.

Who is liable to getting this disease?

  1. Abnormal blistering of skin.
  2. Widespread skin breakdown.
  3. Thickening and hardening of skin on the palms of hands and soles of feet.
  4. Photosensitivity: Includes increased sensitivity to light.
  5. Patchy discoloration of skin.
  6. Feeding difficulties in infancy.
  7. Any traumatic incident related to hands and feet.
  8. Development of dental abnormalities.
  9. Skin ulcer: Burns are formed on the skin from mild ones to blisters.
  10. Gingivitis.
  11. Loss of primary teeth in early ages.
  12. Periodontitis.
  13. Abnormality of the gastric mucosa.

It is an inherited autosomal recessive disorder. This shows that this is transmitted from any of the parents. Children are more liable to getting this disease,as it is more common in infancy. Family history of this disease also has an impact on the sufferer.

How can one diagnose it?

The diagnosis is mainly based on the type of symptoms shown by the patient.The health professional would ask for the genetic history or any traumatic incident. In some cases laboratory tests can be performed to make its diagnosis.

What are the possible available treatments for Kindler’s Syndrome?

This syndrome chiefly affects the skin. So skin should be protected from any kind of exposure.It also affects teeth:

What is the long term outlook for this syndrome?

As this disease causes thinning and blistering on the epidermis of the skin hence it reduces age. The sufferer also somehow becomes photosensitive. Similarly, if it affects the eyelids, it will pose a permanent damage on it.