Bullous Ichthyosis - Infection Question

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abopabop
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by abopabop on Thu Sep 19, 2013 9:33 pm

Bullous Ichthyosis - Infection Question

My son is 10 months old and has Epidermolytic Ichthyosis - Keratin 1. In general his skin condition is pretty well controlled at present using QV cream and ointment.

On the back of his hand he has a circular patch of sore broken weepy skin that alternately blisters and then goes weepy, then dries out and looks like it will heal, but never actually does. His other hand looks fine - the usual flaky skin, but when well moisturised, can look completely normal.

He had a similar persistant patch on his cheek that after several months finally went away when we were prescribed Daktocort. Once the improvement started it was fairly steady improvement, though it did take a number of weeks and we had to use the Daktocort more intensively than usual i.e. 4-5 times a day for a few weeks rather than 1-2 times a day for a maximum of a fortnight.

On his hand we have now, at different times, tried reasonably long courses of Fucidin, Flaminal Forte and Daktocort. None of these has produced an improvement that lasted. The hand has never completely healed, although it does improve to a nearly healed condidtion, but then it blisters and the sore patches re-erupt.

Is there some other infection we should be considering (i.e not staph/strep/yeast) and some other antibiotic/antifungal treatment we could ask for?

Thank you.

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Dr Mary Glover
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by Dr Mary Glover on Fri Sep 20, 2013 9:59 am

Re: Bullous Ichthyosis - Infection Question

With any type of bullous condition the skin will have a tendency to become raw. This is not caused by infection, but by the underlying structural abnormality of the skin. Of course raw areas can become infected, and the infection should be treated, the precise treatment depending on the nature of the infection. Unfortunately treatment of any secondary infection can not prevent the formation of blisters and raw areas. Has your son had specilist nursing input with regard to protection of the skin and application of dressings ?
Dr Mary Glover
Consultant Paediatric Dermatologist

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Dr Mary Judge
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by Dr Mary Judge on Fri Sep 20, 2013 6:02 pm

Re: Bullous Ichthyosis - Infection Question

I agree with Dr Mary Glover's comments. People with BIE do get repeated Staph infections any and everywhere but moreso on face and hands, perhaps because of trauma. This impetigo can lead to more blisters and a bad 'flare'. They can have eczema which itself is generally contaminated with Staph.
and needs steroid cream such as the Daktacort you mentioned.

Non-heaing lesions should be swabbed and the use of antiseptic wash is helpful, eg. Dermol 500 wash (put on skin all over just before bath and wash off in water). Remember the cold sore virus, Herpes Simplex, it can cause recurrent blisters on any part of skin.
Dr Mary Judge
Consultant Paediatric Dermatologist & Consultant Adult Dermatologist

abopabop
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by abopabop on Sun Sep 22, 2013 10:20 pm

Re: Bullous Ichthyosis - Infection Question

Thank you for your responses. I've already tried the tip about putting the Dermol on before putting him in the bath and then washing it off. It certainly worked better than trying to use it once he's in the bath.

We have Polymem dressings and have used them successfully to protect any sore patches left by large blisters that have formed around his nappy waistband. We cut the sticky film off and just use the dressing part.

I suspect Dr. Glover has correctly summarised the problem - that the skin is both infected (or was) but also suffers from being constantly just healing skin and therefore is very raw. If the problem with his hand is now largely due to skin fragility (assuming mostly the infection must be controlled by the creams) then how would you recommend that we protect it? We have, up to now, managed not to stick any dressings to his skin and so securing something to his rather small hand is tricky. Of the Flaminal, Daktocort and Fucidin, which do you think might be expected to be most useful - or which comination?

Any further suggestions very welcome.

abopabop
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by abopabop on Sun Sep 22, 2013 10:22 pm

Re: Bullous Ichthyosis - Infection Question

PS. Is there something you could suggest that would be effective against the Herpes Simplex virus, if that is the problem. We could then talk to our GP and discuss that option.

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Dr Mary Glover
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by Dr Mary Glover on Mon Sep 23, 2013 10:46 am

Re: Bullous Ichthyosis - Infection Question

With regard to which dressings to use this would depend on the state of the skin. I think it would be desirable for him to be assessed by a team specialising in fragile skin disorders in order to determine which dressings would be most suitable.

And with regard to creams - this would depend on the organisms present and the sensitivity or the organisms to antibacterials as assessed by taking a swab. In general it is not advisable to use Fucidin long term as organisms can become resistant to it.

There are treatments for herpes simplex infection but these would only be appropriate if clinical assessment and swab indicate the presence of the virus.
Dr Mary Glover
Consultant Paediatric Dermatologist

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