Bullous Ichthyosiform Erythroderma

Bullous ichthyosiform erythroderma

Bullous ichthyosiform erythroderma appears to be inherited as an autosomal dominant condition.
There is diffuse erythema with scaling and blister formation that is apparent shortly after birth. The bullae usually appear at sites of trauma.

There is slow but steady improvement and by age 10, bullae are not usually a problem.

The scaling continues for life, and indeed, increases with age, causing extensive retention hyperkeratosis in a rippled pattern, particularly accentuated in the flexures, later in life.
There is thickening of the palms and soles. Hair and nails are normal.

Treatment:

  • There is no good treatment for the bullous stage, other than supportive care and the treatment of infection should that arise.
  • The hyperkeratotic phase is improved by oral etretinate or acitretin: but, as such treatment must be continued on a chronic basis, these agents should be started only with circumspection.
Bullous Ichthyosiform Erythroderma

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