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Useful Websites:


British Association of Dermatologists

New Zealand Dermatological Society

Emedicine.com

The Doctor's Doctor

Primary Care Dermatology Society

Patient Info

StatPearls

Dermatology Online Journal

Family Practice Notebook

Australasian College of Dermatologists

UpToDate



Useful Articles:

J Can Dent Assoc 2000.  Clinical Practice.  Oral & Maxillofacial Manifestations of Multiple Sclerosis

American Family Physician 2002.  Auto-Immune Bullous Dermatoses - A Review

Arch Otolaryngol Head Neck Surg 2004.  Linear IgA Disease Presenting as Desquamative Gingivitis.  A Pattern
Poorly Recognized in Medicine

J Can Dent Assoc 2005.  Clinical Practice.  Blistering Mucocutaneous Diseases of the Oral Mucosa - A Review.
Part 1. Mucous Membrane Pemphigoid

Oral Surgery 2009.  Oral mucosal involvement as the sole or main manifestation of Linear IgA Disease - Case
Report & Review of the Literature

ARUP Laboratories 2011.  The Physician's Guide to Lab Test selection & Interpretation - Linear IgA Disease

J Am Acad Dermatol 2011.  Linear IgA Disease limited to the Oral Mucosa

J Genet Syndr Gene Ther 2013.  Chronic Bullous Disease or Linear IgA Dermatosis of Childhood - Revisited

J Clin Aesthet Dermatol 2015.  Linear IgA Bullous Dermatosis - A Rare Clinicopathologic Entity with an Unusual
Presentation

Stomatological Dis Sci 2017.  Dilemmatic Presentation of Linear IgA Disease - A Case Report

Orphanet J Rare Diseases 2019.  Linear IgA Bullous Dermatosis in Adults & Children: A Clinical &
Immunopathological Study of 38 Patients
What is Linear IgA Disease?

Linear Immunoglobulin A (IgA) Disease / Dermatosis is a rare blistering disorder, an auto-immune sub-epidermal
vesiculo-bullous disease
that may be idiopathic or drug-induced.  The clinical presentation is heterogeneous and
appears similar to other blistering diseases, such as
bullous pemphigoid and dermatitis herpetiformis.

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.
Linear IgA Disease
Last Updated 25th October 2020
Photos of Linear IgA Disease
studded with small blisters (vesicles) or large ones (bullæ), 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 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.


What are the causes of Linear IgA Disease?

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, co-trimoxazole, amiodarone,
ciclosporin, 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.


How is it diagnosed?

Linear IgA disease is a sub-epidermal 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, such as  
pemphigus.

A special skin biopsy antibody test,
direct immuno-fluorescence, 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 immuno-fluorescence).  Research
indicates the
antibodies are directed against various basement membrane components (target antigens).


How is it treated?

Most cases have been reported to respond to dapsone or sulfapyridine.

Some clinicians favor the use of
sulfapyridine because of the lower incidence of adverse effects.  However, some
patients' conditions may not respond to
sulfapyridine but do respond to treatment with dapsone.  A response may
be seen in 48 - 72 hours.

Other reportedly useful medications include
prednisolone, sulfamethoxypyridazine, colchicine, dicloxacillin,
mycophenolate mofetil & intravenous immunoglobulin.

Drug-induced disease may be treated merely by withdrawal of the offending agent.  In cases of
linear IgA
dermatosis
induced by vancomycin, new lesions stop forming within approximately 2 weeks of withdrawal.

Particularly severe cases of drug-induced
linear IgA dermatosis respond to a short course of oral corticosteroids.

Although the condition may eventually be cured, many patients require long-term treatment as a reduction in dose
of medication results in further blistering.



Differential Diagnosis:

A number of skin conditions have an almost identical appearance. These include: