The Experts below are selected from a list of 1989 Experts worldwide ranked by ideXlab platform

Alex Reyentovich - One of the best experts on this subject based on the ideXlab platform.

Hiroko Kanda - One of the best experts on this subject based on the ideXlab platform.

Todd S. Cutler - One of the best experts on this subject based on the ideXlab platform.

Paola Grammatico - One of the best experts on this subject based on the ideXlab platform.

  • palmoplantar keratoderma pseudo ainhum and universal atrichia a new patient and review of the palmoplantar keratoderma congenital alopecia syndrome
    American Journal of Medical Genetics Part A, 2010
    Co-Authors: Marco Castori, Michele Valiante, Marco Ritelli, Nicoletta Preziosi, Marina Colombi, Mauro Paradisi, Paola Grammatico
    Abstract:

    Palmoplantar keratoderma (PPK) may concur with congenital alopecia (CA) in various genodermatoses. We report on a 10-year-old girl with generalized atrichia and a severe form of PPK causing pseudo-ainhum, Sclerodactyly, and contractures, a phenotype not consistent with any well-defined condition. Non-specific additional findings comprised mild nail dystrophy and widespread keratosis pilaris including ulerythema ophryogenes. Direct sequencing of the GJB2 and LOR coding regions yielded normal results. A review identified two additional sporadic and four familial cases with PPK and CA. Comparison between familial cases suggested the existence of two genetically and phenotypically distinct types of PPK-CA: (i) an autosomal dominant form (Stevanovic type), a variable and benign phenotype without significant hand complications, and (ii) a more complex autosomal recessive variant (Wallis type) with contractures, Sclerodactyly, and pseudo-ainhum. Nuclear cataract may represent an additional although not constant finding in the Wallis type PPK-CA. Further reports are required to test this preliminary conclusion.

  • Palmoplantar keratoderma, pseudo‐ainhum, and universal atrichia: A new patient and review of the palmoplantar keratoderma‐congenital alopecia syndrome
    American Journal of Medical Genetics Part A, 2010
    Co-Authors: Marco Castori, Michele Valiante, Marco Ritelli, Nicoletta Preziosi, Marina Colombi, Mauro Paradisi, Paola Grammatico
    Abstract:

    Palmoplantar keratoderma (PPK) may concur with congenital alopecia (CA) in various genodermatoses. We report on a 10-year-old girl with generalized atrichia and a severe form of PPK causing pseudo-ainhum, Sclerodactyly, and contractures, a phenotype not consistent with any well-defined condition. Non-specific additional findings comprised mild nail dystrophy and widespread keratosis pilaris including ulerythema ophryogenes. Direct sequencing of the GJB2 and LOR coding regions yielded normal results. A review identified two additional sporadic and four familial cases with PPK and CA. Comparison between familial cases suggested the existence of two genetically and phenotypically distinct types of PPK-CA: (i) an autosomal dominant form (Stevanovic type), a variable and benign phenotype without significant hand complications, and (ii) a more complex autosomal recessive variant (Wallis type) with contractures, Sclerodactyly, and pseudo-ainhum. Nuclear cataract may represent an additional although not constant finding in the Wallis type PPK-CA. Further reports are required to test this preliminary conclusion.

Ken Hamasaki - One of the best experts on this subject based on the ideXlab platform.