Epidermolysis Bullosa

We evaluate biopsies for the diagnosis of epidermolysis bullosa with electron microscopy and with immunomapping of the basement membrane zone. We provide this service in close collaboration with Drs. Latanya Benjamin, Phuong Khuu and Thomas Leung, who direct the pediatric dermatology service at Stanford and with Dr. Peter Marinkovich , director of the clinic for bullous disorders.

It is paramount that diagnostic biopsies will be performed on a freshly induced blister. This is best achieved by rotating a pencil eraser on intact skin. Please perform 2 punch biopsies (4 mm) at the edge of the blister, containing about 1/3 lesional and 2/3 perilesional skin. Please do not bisect the punch biopsies. The skin in EB patients is usually so fragile that bisecting a punch biopsy will denude the epidermis and render the specimen useless. Please do not select an existing blister, even if it appears fresh, clinically. Early reepitheliazation will prevent us from establishing the level of the split.

Please submit one biopsy in Zeus or Michels fixative for immunomapping and the second biopsy in Glutaraldehyde for EM. No H&E is necessary unless you suspect an autoimmune blistering disorder. Include clinical history and clinical findings.

Download requisition sheet

Our antibody panel for mapping of the basement membrane zone includes:

mA6K5, anti-keratin 5
mAb K14, anti-keratin 14
mAb HD121, anti-plectin
mAb 123, collagen XVII (BP180)
mAb 3E1, anti-beta 4 integrin
mAb K140, anti-laminin-332 beta 3 chain
mAb D4B5, anti-laminin-332 gamma 2 chain
mAb BM165, anti-laminin-332 alpha 3 chain
mAb AE2, anti-type IV collagen
mAb LH-72, anti-type VII collagen

Primary panel: Antibodies against collagen XVII (BP180), collagen IV, collagen VII, laminin-332 gamma 2 chain

Secondary panel: Antibodies against laminin-332 beta 3 chain, laminin-332 alpha 3 chain, and beta 4 integrin

Antibodies against keratin 5, keratin 14, and plectin are run on an as-needed basis.