Test Directory

• Histopathological evaluation performed by Board Certified Pathologist.
• Includes: Anti-IgG, Anti-IgA, Anti-IgM, Anti-C3, Antifibrinogen, C3.

• Detect molecules such as immunoglobulins, complement components, fibrinogen or immune complexes in biopsy specimen.
• May assist in confirming a diagnosis of bullous pemphigoid, cicatricial pemphigoid, other variants of pemphigoid, all types of pemphigus, dermatitis herpetiformis, linear IgA bullous dermatosis, chronic bullous disease of childhood, epidermolysis bullosa acquisita, porphyria cutanea tarda, bullous eruption of lupus erythematosus, herpes gestationis, systemic lupus erythematosus, discoid lupus erythematosus, or other variants of lupus erythematosus, vasculitis, lichen planus, and various other inflammatory diseases.

Alternate Specimen:

Min. Volume External: 4 mm skin bunch biopsy is optimal

Sources: Skin, Oral Mucosa, Conjunctiva, Esophagus

Volume External:

Alternate Container:

Collect specimen in vial containing Zeus transport media. DIF bottles are available from HNL Lab Medicine 484-425-8166.

Obtaining biopsies:

• Use sharp 4 mm punch instrument
• Immediately place biopsy into transport media.

Biopsy Site:

Guidelines/Optimal sites for acquiring Direct Immunofluorescence

1. Pemphigus and Pemphigoid groups: (includes Epidermolysis Bullosa Acquisita, Linear IgA Bullous Dermatosis and Chronic Bullous Disease of Childhood):

• Biopsy erythromatous perilesional skin or mucosa or the edge of active blister. Label as perilesional skin/mucosa.

2. Cicatricial Pemphigoid:

• perilesional skin, mucosa or conjunctiva

3. Dermatitis Herpetiformis:

• Biopsy normal-appearing skin approximately 3 mm from the lesional/blistered skin. Label as perilesional skin. Multiple biopsies may be required for diagnosis. False negative DIF can occur if lesional skin is biopsied.

4. Connective Tissue Disease:

• For Lupus Erythematous (LE) and Dermatomysitis(DM):
• For DLE, SCLE and DM: Biopsy involved area of the skin (erythematous or active borders of an established lesion which is > 3 months is preferred site to confirm discoid or systemic lupus.) Label as involved lesion. Avoid ulcers, old lesions and facial lesions, if possible.
• For SLE and the Lupus Band Test (LBT): Perform two biopsies. Biopsy erythematous or active border of established lesion (involved/lesional skin.) Biopsy uninvolved, non-exposed (non-lesional and sun-protected) skin to detect a lupus band. Suggested anatomic sites include buttock or medial thigh. Label as uninvolved non-exposed skin.
• For Bullous SLE: Perilesional skin present 6-8 weeks, but actively inflamed.

5. Mixed Connective Tissue Disease:

• Biopsy similar to lupus erythematous. If sclerodermoid features present, biopsy inflamed area. Label as involved or uninvolved, exposed or non-exposed.

6. Vasculitis and Urticaria:

• Biopsy the erythematous or active border of a new lesion (ideally < 24-48 hours old.) Avoid old or ulcerated lesions and when possible distal extremities. Label as involved skin. If active lesion not available, uninvolved skin may yield diagnostic information, but is less sensitive.

7. Porphyria Cutanea Tarda and Pseudoporphyria:

• Biopsy involved skin at the edge of an active blister. Avoid old lesions and ulcers. Label as involved skin.

8. Lichen Planus and Lichenoid Reactions:

• Biopsy involved skin. Avoid old lesions and ulcers. Label as involved skin.

• Submit specimen with an EPIC order, Tissue Pathology Requisition Form (HNL-31), or one of the laboratory's electronic forms.
• Include the following pertinent information (age, gender, clinical diagnosis, anatomic biopsy site, exposure of site to sun (exposed versus unexposed), and if tissue is lesional, perilesional (edge of active lesion), or uninvolved normal appearing skin or mucosa.
• Label vial with patient's name, medical record or date of birth, biopsy site and date.
• Seal container tightly.
• Testing CANNOT be performed on tissue that has been fixed in formalin.
• If more than one biopsy is obtained at different sites, each specimen requires a seperate container and a separate order. Otherwise, tissue will be processed and reported as one specimen.
• If light microscopic histopathologic correlation is desired, collect second biopsy of lesional skin and submit in formalin for paraffin embedded tissue processing (See Histopathology, Skin Biopsy, Light Microscopy)

Testing CANNOT be performed on tissue that has been fixed in formalin.