Pachyonychia Congenita: Electron Microscopic and Epidermal Glycoprotein Assessment Before and During Isotretinoin Treatment

摘要: • Two patients, a father and son, with pachyonychia congenita were treated orally administered isotretinoin because the extreme deformity discomfort associated their massive keratoderma interfered work school, respectively. While clinical benefits could not be sustained, electron microscopic findings compatible suppression of abnormal keratinization observed. In addition, skin biopsy samples analyzed by sodium dodecyl sulfate—polyacrylamide gel electrophoresis, gels then subjected to lectin overlay technique concanavalin A labeled iodine 125. The distribution specific glycoproteins was found different for lesional as against normal epidermis. procedure repeated after oral treatment isotretinoin. glycoprotein pattern epidermis clearly distinguishable from both pretreatment epidermis; it mostly intermediate between two. virtually unaffected retinoid treatment. (Arch Dermatol1984;120:1475-1479)