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Old 02-19-2004, 12:24 PM   #1
irish34
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Join Date: Nov 2003
Location: Milwaukee, WI
Posts: 154
Question Childhood Linear IgA Disease

My 3 and a half year old daughter was just diagnosed with Linear IgA via skin biopsy. We have been dealing with misdiagnosis since June 2003. There are going to start treating it with Erythromycin and a topical steroid creme. Has anyone dealt with this. It is so rare. The Childrens Hospital here said that they only see 1 case of this a year. 1 a year! I can't believe it!
Anyone with any information or stories would be so much appreciated!
Thank you!!!
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Old 02-20-2004, 12:16 PM   #2
irish34
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Re: Childhood Linear IgA Disease

Please, anyone that has ANY information about this, I'd really like to hear about it. There is not much information out there other than clinical data. Which I don't understand the half of.
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Old 02-23-2004, 01:00 PM   #3
CheerAngel
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Wink Re: Childhood Linear IgA Disease

Hi there, I'm so sorry that your child has gotten this diease. I have found some info for you and hope it helps. If you don't understand, ask me, I will try and see if I can answer them for you. good luck!

=Linear IgA Disease=
Linear IgA Disease is a rare blistering disorder. It is nearly identical to a similar condition that affects children, chronic bullous disease of childhood.

Chronic bullous disease of childhood usually presents before puberty with an abrupt onset of blistering in the genital region, later affecting hands, feet and face. In adults with linear IgA disease, the limbs are more often the first sites, although any area of the body may be affected later.

=Clinical features=
Clear round or oval blisters may arise from normal-looking or red skin. Red flat or elevated patches may arise, studded with small blisters (vesicles) or large ones (bullae), often target-shaped. The tendency for new blisters to arise in a ring around an old one is called the string of beads sign, and groups of small blisters may be described as a cluster of jewels. Crusts, scratch-marks, sores and ulcers may arise. The lesions can resemble other uncommon blistering skin diseases especially erythema multiforme, bullous pemphigoid and dermatitis herpetiformis.

The intensity of itching is variable. Blisters and ulceration on the lips and inside the mouth affect about 50%. Eye involvement may result in irritation, dryness, light sensitivity, blurred vision, corneal scarring and even blindness.

=Cause=
Linear IgA disease is usually idiopathic i.e. it arises spontaneously. However, it sometimes follows infection and is rarely caused by drug allergy. Vancomycin is the most frequently associated drug, although diclofenac, captopril, cotrimoxazole, amiodarone, cyclosporin, glibencamide, lithium, penicillin, cefamandole, phenytoin, sodium hypochlorite, and somatostatin have been implicated in case reports. Drug-induced disease resolves with withdrawal of the offending agent. Linear IgA disease has also been rarely associated with lymphoma, haematological conditions, rheumatological conditions, ulcerative colitis and solid tumours.

=Biopsy findings=
Linear IgA disease is a subepidermal blistering disorder. This means that a skin biopsy reports blistering just under the epidermis as opposed to some blistering disorders that result in blistering within the epidermis eg pemphigus.

A special skin biopsy antibody test, direct immunofluorescence, reveals the immunoglobulin IgA along the basement membrane of the epidermis in a linear pattern. Sometimes these IgA antibodies can be detected by a blood test (indirect immunofluorescence). Research indicates the antibodies are directed against various basement membrane components (target antigens).

~go to this page [url]www.dermnetnz.org/index.html[/url] and search under Patient Information for Linear IgA disease.
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Old 02-24-2004, 12:27 AM   #4
irish34
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Location: Milwaukee, WI
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Re: Childhood Linear IgA Disease

CheerAngel--
Thank you so much for all the useful information--are you familiar with this disease?!? I am wondering whether she just has the childhood form, or what? The biopsy was positive for IgA, but the Derma said she'd likely grow out of it.
This is so confusing and heartbreaking! Do you know of someone with this?
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Old 02-24-2004, 12:30 PM   #5
CheerAngel
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Talking Re: Childhood Linear IgA Disease

Hi there, it's my pleasure to be able to help you. If you need anymore help, let me know! Your child might be able to out grow this disease and I hope she does! I don't know of someone with this problem and it is the first time I heard from you. So I'm not very professional with this disease. But I thought it would be good if I could help you. I personally have some skin problems that not alot is known about it, and I know the feeling of not knowing more about something. All the best and hope to hear from you!
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