Keratosis extremitatum progrediens

Keratosis Extremitatum Progrediens is a rare skin disorder characterized by the progressive thickening of the skin on the extremities. This condition is part of a group of diseases known as keratodermas, which affect the palms and soles by causing abnormal skin growth and thickening. Keratosis Extremitatum Progrediens is distinguished by its pattern of spread and the areas of the body it affects.

Symptoms and Diagnosis

The primary symptom of Keratosis Extremitatum Progrediens is the gradual development of thick, hard skin on the hands and feet. This can lead to discomfort, reduced mobility, and in severe cases, pain. The condition may start in childhood or adolescence and tends to progress over time.

Diagnosis of Keratosis Extremitatum Progrediens involves a thorough examination of the affected skin, patient history, and sometimes genetic testing. Since it is a rare condition, diagnosis can be challenging and often requires consultation with a specialist in dermatology.

Causes

The exact cause of Keratosis Extremitatum Progrediens is not well understood, but it is believed to have a genetic component. Mutations in specific genes related to skin growth and development may play a role in the development of this condition. It is thought to be inherited in an autosomal dominant manner, meaning only one copy of the altered gene is necessary to cause the disorder.

Treatment

There is no cure for Keratosis Extremitatum Progrediens, and treatment focuses on managing symptoms and improving the quality of life for those affected. Treatment options may include:

• Keratolytic agents to soften and remove thickened skin
• Moisturizers and emollients to keep the skin hydrated
• Physical therapy to maintain mobility in the hands and feet
• In severe cases, surgery may be considered to remove excessively thickened skin

Prognosis

The prognosis for individuals with Keratosis Extremitatum Progrediens varies. While the condition is not life-threatening, it can significantly impact quality of life. Early and ongoing treatment can help manage symptoms and prevent complications.

See Also

• Palmoplantar Keratoderma
• Skin Disorders
• Genetic Disorders