XXIII. Über das seltene Syndrom von kongenitalen Keratosen an Haut und Kornea

Pachyonychia congenita (Jadassohn-Lewandowsky Syndrome)

“…In the ophthalmologic literature, it is also known as Schafer's syndrome [6]. At present, many authors share the views of Kumer and Loos [7], namely that JLS appears in one of the following three clinical forms: (1) pachyonychia, palmoplantar keratoderma, follicular hyperkeratosis; (2) pachyonychia, palmoplantar keratoderma, follicular hyperkeratosis, leukokeratosis of the tongue and buccal mucosa, and (3) the third form includes the same symp toms as quoted under 2 as well as corneal dyskeratosis or cataract [8]. A number of authors described further anomalies as blisters and changes in pigmentation [3], hypotrichosis and other hair growth deficiencies [9], dental disorders [10,11], mental retardation [12,13] and others.…”