Jadassohn Lewandowsky syndrome: Type 1 pachyonychia congenita

PC is commonly described as a rare genodermatosis characteristically manifesting as massive subungual hyperkeratosis with nail thickening, focal palmoplantar keratoderma alongwith deep f issur ing and blistering, oral leukokeratosis and discrete follicular hyperkeratosis [1,2]. Other features including abnormalities of teeth, hairs and larynx can also be seen depending on the clinical types. Onset is usually within first year of life and mode of inheritance is autosomal dominant. After first description by Muller in 1904, next reports were published by Wilson in 1905 and Jadassohn Lewandowsky in 1906 [3-5]. Further, depending on the genetic mutation and clinical correlations, 4 types of PC have been defined.