Stains - Collagen XVII

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Stains

Collagen XVII

Authors: Kara Hamilton, M.S., Nat Pernick, M.D., PathologyOutlines.com, Inc.

Revised: 22 September 2012, last major update March 2010

General

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● Collagen XVII (BP180) is a transmembrane component of hemidesmosomes responsible for mediating epidermal adhesion to the dermal layer (OMIM 113811)

Terminology

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● Also called COL17, bullous pemphigoid antigen 2 (BP antigen II) and BP180

Pathophysiology / Clinical

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● Mediates epidermal adhesion to the dermal layer (Dermatol Clin 2010;28:61); mutations / deficiency are associated with non-Herlitz junctional epidermolysis bullosa (J Invest Dermatol 2005;125:1112, OMIM 226650) and abnormal tooth formation of amelogenesis imperfecta (Int J Mol Med 2006;18:333)

● Autoantibodies are associated with bullous pemphigoid, herpes gestationis and cicatricial pemphigoid (J Invest Dermatol 1996;106:141, Am J Pathol 2000;156:685, J Invest Dermatol 1997;109:573)

● Regulates ameloblast differentiation; mutations are associated with enamel hypoplasia (Am J Pathol 2009;174:91)

● Lost during normal keratinocyte maturation but reexpressed upon malignant transformation (Appl Immunohistochem Mol Morphol 2008;16:433)

● May play a role in trophoblast invasion, and therefore a role in placental formation

Drawings, University of Oulu, Finland

Positive stains (normal)

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● Stratified and complex epithelia such as skin, mucous membranes, eye

● First trimester and term syncytial and cytotrophoblastic cells of normal placenta and hemidesmosomes of amniotic epithelium (Matrix Biol 2008;27:190)

Positive stains (disease)

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● Invasive front of cutaneous squamous cell carcinoma (J Histochem Cytochem 2003;51:921)

Micro images

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Fig A, part a: Keratinocytes

Patients with inherited epidermolysis bullosa

Fig 4a: lack of Collagen XII staining

Fig 4b: partial lack of staining adjacent to normal skin with intense staining

Additional references

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References: Wikipedia

End of Stains > Collagen XVII

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