Pachyonychia congenita typ II (Jackson-Lawler syndrome)

Pachyonychia congenita (PC) is a rare ectodermal dysplasia with variable ex pression. The condition is usually inherited as an autosomal dominant trait . Several classifications of PC have been proposed. Feinstein and colleague s suggested four clinical types of PC. Type II, the Jackson-Lawler-Syndrome , is characterized by multiple epidermal cysts, palmoplantar bullae and hyp erhidrosis as well as natal teeth in addition to the main findings of pachy onychia, palmoplantar hyperkeratosis and follicular keratosis. We report tw o patients (father and son) with Jackson-Lawler-Syndrome and describe in de tail pathogenesis, diagnostic criteria and treatment approaches as well as the different classifications of pachyonychia congenita.