Test Code #

24

CPT Code(s) #

88346

Test Name

Linear IgA, Bullous Dermatosis of Childhood, Ab Titer - IgA

Disease Name:

Linear IgA Bullous Dermatosis (LABD)

Type of Study:

Serum Studies

Methodology:

Indirect Immunofluorescence

Substrate:

Normal and Split Human Skin

Reference Range:

Negative: <1:5

Units:

Titer

Schedule:

Assay performed daily Monday-Friday. Report availability is within 48 hours from the time of specimen receipt

Specimen Requirements:

Collect 5-10 ml of blood in a red top or serum separator tube. If possible, separate serum from clot and place into red capped tube provided with Beutner Laboratories collection kits. If separation facilities are not available, the blood can be sent in the tube used for collection.

Sample Stability:

Stable at ambient temperature during shipment. If sample is stored prior to shipment, it is stable refrigerated (2-8ºC) up to five days and frozen (-20ºC or lower) up to one year.

Clinical Relevance:

Linear IgA Bullous Dermatosis (LABD) is a chronic, acquired, autoimmune blistering disease. It is characterized by subepidermal blistering and linear deposition of immunoglobulin A (IgA) basement membrane antibodies. The disease affects both children and adults. There are some differences in their clinical presentations, therefore the disease in children is termed as Chronic Bullous Disease of Childhood (CBDC). The immunopathology of LABD and CBDC is similar (Venning, 2011). IgA antibodies to BMZ of skin by IIF on split human skin can be detected in about 30% adult patients with LABD and in almost 75% children with CBDC. This test helps to differentiate the autoantibodies reacting on the epidermal roof of the split (target antigens are the shed ectodomains of BP180 molecule) from the antibodies reacting on the floor (target antigen is LAD285 and in rare cases type VII Collagen). Patients with LABD may have co-existing IgG antibodies, therefore IIF test for IgG and IgG4 antibodies on Split Human Skin (#014) should also be done. Vanessa A. Venning. Linear IgA Disease: Clinical Presentation, Diagnosis, and Pathogenesis. Dermatologic Clinics, 2011;29 (3): 453-458.

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