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21 Month Old With Epidermolysis Bullosa


QUESTION

My 21 month old grandson was born with a rare skin condition called Epidermolysis Bullosa or EB for short. The type is Dowling meara, simplex. It is a genetic disease and a cure has not been found as yet. I was wondering if you have any suggestions. The blisters are more prevalent on hands and feet and the baby has difficulty walking.


ANSWER

Epidermolysis Bullosa is a group of diseases characterized by blister formation after minor injury to the skin. This family of inherited disorders range in degree of severity from mild to very serious and disabling.

Daily skin care is a mainstay of treatment in EB, with avoidance of trauma a primary goal. Management of skin infections is an important part of EB patient care. Systemic antibiotics are frequently used for cutaneous infection and chronic wound colonization is managed best by regular cleansing and bathing in modified Dakin's solution and topical antibiotics as needed. Modified Dakin's solution is 2 teaspoons of Clorox per gallon of bath water.

In general, a good anti-oxidant based vitamin/mineral formula would be a very wise move, as many BE patients are nutrient-deficient (1), and the basis of the blistering reactions are related to excessive free radical activity. This is particularly true of vitamin E. (2,3)

1. Birge K. utrition management of patients with epidermolysis bullosa. J Am Diet Assoc. 1995 May;95(5):575-9.

2. Pehr K, Forsey RR. Why don't we use vitamin E in dermatology? CMAJ. 1993 Nov 1;149(9):1247-53.

3.Ayres S Jr. Epidermolysis bullosa controlled by vitamin E. Int J Dermatol. 1986 Dec;25(10):670-1.




The Ask Dr. D'Adamo internet advice column ran from 1996 to 2009, at which time Dr. D'Adamo's teaching and programming responsibilities no longer allowed him to devote time and resources to directly answering visitor questions. However we've recently reorganized this treasure-trove of material and made it again available to his readership. He occasionally posts new entries. These are marked with a NEW tag.



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