In 1906 Jadassohn and Lewandowsky1 described in "Ikonographia dermatologica" a cutaneous rarity that they believed to be an unclassified dermatosis, a disease sui generis to which they gave the name pachyonychia congenita. This original paper had the subtitle "Keratosis Disseminata Circumscripta, Tylomata and Keratosis Linguae." A review of the earlier literature reveals nothing apparently similar, and though the entity is one with a definite clinical picture it is not well known, probably because it is infrequently mentioned in the standard dermatologic works. Only a few reports of genuine cases of pachyonychia congenita can be found in the American literature. The cases described by Howard Fox2 in 1928, by Andrews3 in 1929 and by Diazo4 in 1934 fulfil the clinical requirements of the disease. Perhaps cases are occasionally encountered in every clinic that pass undiagnosed, but in the available American literature these three reports afford
SOHRWEIDE AW. PACHYONYCHIA CONGENITA: REPORT OF A CASE. Arch Derm Syphilol. 1935;32(3):370–376. doi:10.1001/archderm.1935.01470030012002
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