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November 1984

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

Author Affiliations

From the Department of Dermatology, University of Texas Medical Branch, Galveston (Drs Thomas, Jorizzo, and Brysk and Ms Miller); the Departments of Dermatology and Pathology, Baylor College of Medicine, Houston (Dr J. Tschen); and the Division of Dermatology, Department of Internal Medicine, University of New Mexico School of Medicine, Albuquerque (Dr E. Tschen).

Arch Dermatol. 1984;120(11):1475-1479. doi:10.1001/archderm.1984.01650470081018

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

(Arch Dermatol 1984;120:1475-1479)