Reproduction of the characteristic morphologic changes of familial benign chronic pemphigus in cultures of lesional keratinocytes onto dead deepidermized dermis.
We report the in vitro reproduction of the classic histologic and ultrastructural features of familial benign chronic pemphigus (FBCP) by seeding suspensions of lesional keratinocytes onto healthy heterologous dead deepidermized dermis (DED). With the use of normal keratinocytes, control cultures showed a well-differentiated epidermis, with keratohyaline granules and lamellar bodies. To the best of our knowledge this is the first successful culture of FBCP with the use of dispersed lesional keratinocytes. The results suggest that in FBCP the epidermis is the site of the defect leading to acantholysis, without any dermal contribution.
De Dobbeleer
G
G
Department of Dermatology, Hôpital Erasme, Université Libre de Bruxelles, Belgium.
De Graef
C
C
M'Poudi
E
E
Gourdain
J M
JM
Heenen
M
M
D002455:Cell Division; D002478:Cells, Cultured; D002908:Chronic Disease; D003470:Culture Media; D004848:Epithelium / Q000502:physiology; D006801:Humans; D015603:Keratinocytes / Q000502:physiology* / Q000648:ultrastructure; D010392:Pemphigus / Q000473:pathology*; D012879:Skin Physiological Phenomena
D003470:Culture Media