Author/Authors :
Mohammadi, Saman Department of Dermatology - Leishmaniasis Research Center - Kerman University of Medical Sciences, Kerman , Khalili, Maryam Department of Dermatology - Leishmaniasis Research Center - Kerman University of Medical Sciences, Kerman , Fadai, Fahameh Department of Dermatology - Leishmaniasis Research Center - Kerman University of Medical Sciences, Kerman , Badakhsh, Hoda Department of Dermatology - Leishmaniasis Research Center - Kerman University of Medical Sciences, Kerman , Farajzadeh, Saeedeh Department of Dermatology - Leishmaniasis Research Center - Kerman University of Medical Sciences, Kerman , Mohebbi, Azadeh Department of Dermatology - Leishmaniasis Research Center - Kerman University of Medical Sciences, Kerman , Aflatoonian, Mahin Department of Dermatology - Leishmaniasis Research Center - Kerman University of Medical Sciences, Kerman
Abstract :
Goltz syndrome or focal dermal hypoplasia is a rare syndrome
with mesoectodermal hypoplasia. This syndrome is an X-linked
dominant disorder with involvement of the cutaneous, ocular,
dental and skeletal systems. The most significant feature of
this disease is connective tissue dysplasia. Here, we report a
30-year old woman who presented with congenital unilateral
linear atrophic areas on her trunk, back, and upper and lower
extremities. She has 3 daughters, 2 of whom had the same skin
lesions that were consistent with Goltz syndrome. Skin lesions in
the younger daughter were more severe than her older sister and
mother. The younger daughter had multiple bone deformities in
the form of clinodactyly and lobster-claw malformation. She also
had nail dystrophy of her fingers and umbilical herniation. The
older daughter only had syndactyly and skin atrophic lesions.
Keywords :
Goltz syndrome , Goltz syndrome , clinodactyly , hypoplasia
