Generalized epidermolytic ichthyosis with palmoplantar hyperkeratosis

Infantile epidermolytic ichthyosis with prominent maternal palmoplantar keratoderma.

Epidermolytic Ichthyosis (EI) is a rare autosomal dominant genodermatosis. Although an inherited disorder, 50% of cases represent novel mutations. This disorder presents as a bullous disease in newborns progressing to a lifelong ichthyotic skin disorder.  Other manifestations include palmoplantar keratoderma (PPK).  EI results from mutations in the keratin 1 and keratin 10 genes. Phenotypic var...

Epidermolytic hyperkeratosis with rickets.

A 6-year-old child presented with generalized hyperkeratosis, most marked over the flexures; windswept deformity of the legs; and limping since 3 years. On the basis of the clinical, histopathologic and biochemical findings, he was diagnosed as a case of epidermolytic hyperkeratosis with rickets. He was treated with parenteral vitamin D3 and calcium supplements orally. Nutritional rickets has b...

Epidermolytic Hyperkeratosis report

A 20 year old boy presented to the dermatology department for treatment of a congenital icthyosis with a history of generalized erythroderma and trauma related blistering since birth. At the time of presentation he was noted to have red hyperkeratotic plaq corrugated over the joint flexures, elbows, knees after birth erythema and blistering improved but patient developed hyperkeratotic scaling ...

Epidermolytic Hyperkeratosis - case report*

Epidermolytic hipercetarose is a rare genodermatosis, with a prevalence of 1:100.000 to 1:300.000, with autosomal dominant inheritance. We report the case of a 5 year old girl who presented an hypertrophic verrucous plaques in the neck, under arm, buttocks, knees, pelvis, legs, dorsum of the right foot and elbows. Histological examination of the skin lesions showed typical changes of epidermoly...

Antibiotic therapy in a boy affected by generalized epidermolytic hyperkeratosis.