Definition
Definition
Genodermatosis sclerosatrophicans keratoderma of the extremities is a palmoplantar keratoderma that appears from birth in a diffuse form, and whose thickness is less significant than for other palmoplantar keratodermas.
It is a familial, dominant and autosomal hereditary condition (it is sufficient for one of the two parents to carry the genetic anomaly on a non-sex chromosome for the offspring to have the disease).
Symptoms
Symptoms
Symptoms of keratodermic sclero-atrophic genodermatosis of the extremities are:
- This skin disease presents with well-circumscribed limits and predominates mainly at the pressure point.
- La keratoderma is discreet, more palmar than plantar (observed much higher on the palms of the hands than on the soles of the feet).
- It is accompanied by a sclerodactyly, which is characterized by a progressive skin condition localized to the fingers, characterized by thickening of the skin, subcutaneous tissues, and sometimes deep tissues which harden, then atrophy, and ulcerate.
- The disease can then, in some patients, affect the arms, feet, legs, trunk and face.