Pachyonychia Congenita

Resistant Erosive Lesions in Pachyonychia Congenita of Jadassohn

“…The purpose of this paper is not to report a case of pachyonychia congenita of Jadassohn, which has been excellently described by Tauber, Goldman and Claassen, 1 Sohrweide,2 Andrews and Strumwasser 3 and Diasio,4 or to summarize the thorough reports of Hammett and Reimann 5 and Brunsting and Simonsen 6 on the sulfhydryl groups, but to demonstrate the value of buffered cysteine hydrochloride in other conditions than ulcerated lesions. I wish to report here the progress of a girl, aged 20, previously presented by Tauber and his associates.…”

Pachyonychia Congenita (Jadassohn and Lewandowski)

“…The other chief lesions which as a rule appear soon after birth, are leucokeratosis of the tongue, symmetrical follicular keratoses affecting the extensor surfaces, keratoses of the palms and soles, and bulle on the feet. A number of other symptoms have been described, notably hyperidrosis of palms and soles, herpetiform lesions round the mouth, dyskeratosis of the cornea with opacities [13], keratosis of the buccal mucosa as well as the tongue, and in one case involvement of the nasal mucosa and tympanic membranes [2]. The onset of these subsidiary symptoms may take place at any time after birth, sometimes many years later.…”