Transgrediens et progrediens palmoplantar keratoderma

Transgrediens et progrediens palmoplantar keratoderma (PPK) is a rare inherited skin disorder characterized by marked thickening of the skin on the palms of the hands and soles of the feet (palmoplantar keratoderma) that extends beyond the palmoplantar margins onto the dorsal surfaces of the hands and feet (transgrediens) and progressively spreads to adjacent areas over time (progrediens). This condition is also known as Greither disease or Greither syndrome, named after the physician who first described it. The keratoderma typically presents in early childhood and tends to worsen with age, sometimes extending onto the wrists, Achilles tendon area, and knees. The disease primarily affects the integumentary system (skin). Key clinical features include diffuse, symmetric thickening of the palms and soles with a characteristic spreading pattern beyond the normal palmoplantar boundaries. Patients may also develop hyperhidrosis (excessive sweating) of the affected areas, which can lead to secondary fungal infections and malodor. The thickened skin can cause significant discomfort, reduced hand dexterity, and difficulty walking. Some patients may also exhibit mild involvement of other keratinized surfaces. Greither disease has been associated with mutations in the keratin gene KRT1 and in some cases other genes involved in epidermal differentiation. Treatment is primarily symptomatic and supportive. Topical keratolytic agents such as salicylic acid, urea-based creams, and topical retinoids are commonly used to reduce skin thickness. Oral retinoids (such as acitretin) may be prescribed in more severe cases. Regular mechanical debridement by a dermatologist or podiatrist can help manage the thickened skin. There is currently no curative treatment, and management focuses on improving quality of life and maintaining functional use of the hands and feet.

Common questions about Transgrediens et progrediens palmoplantar keratoderma