Hopf Verruciform Acrokeratosis

What is Hopf's verruciform acrokeratosis?

Hopf's acrokeratosis verruciformis is a rare genodermatosis classified as a disorder of keratinization. It is often considered a variant of Darier disease. It was first identified in 1931 by Dr. Gustav Hopf, a German dermatologist.

What is the cause of Hopf's acrokeratosis verruciformis?

Hopf's acrokeratosis verruciformis is due to an abnormality ATP2A2 gene. Has autosomal dominant inheritance, meaning that only one copy of the affected gene needs to be inherited to develop the disease. If one parent is affected, the chance of a child developing Hopf's acrokeratosis verruciformis is 1 in 2 (50%).

Mutations in ATP2A2 They also cause Darier disease. The gene encodes the calcium sarcoplasmic reticulum ATPase 2 pump, controlling intracellular calcium. It is presumed that the impaired operation of this pump alters the capacity of desmosomes to link keratinocytes together.

What are the clinical characteristics of Hopf's verruciform acrokeratosis?

Hopf's verruciform acrokeratosis is characterized by multiple, flat-topped, symmetricalskin color keratotic injuries to the back of the hands and feet. They are usually present from birth or early childhood, but occasionally emerge in adult life. Other features may include:

• grouped or isolated papules on the knees, elbows and forearms
• Holes in the palms of the hands and soles of the feet.
• A dystrophy: Pearl white nailor nails with longitudinal ridges and nicks on the free edge

The skin changes in Hopf's verruciform acrokeratosis are asymptomatic and rarely lead to any complications.

How is Hopf's acrokeratosis verruciformis diagnosed?

Hopf's acrokeratosis verruciformis is diagnosed clinically. This is easier if there are family members with similar skin findings. Sometimes a skin biopsy It is done to help with diagnosis.

The key features in histology They are hyperkeratosis, acanthosis, papillomatosis and a thickened granular layer. Rarely, acantholysis is seen. Circumscribed epidermal elevations, known as 'church spires' are a distinctive find. See Darier disease pathology.

What is the treatment for Hopf's acrokeratosis verruciformis?

Since Hopf's verruciform acrokeratosis is asymptomatic and located, no treatment is required. If a patient prefers treatment for aesthetic reasons, destruction of the papules can be performed with cryotherapy or a destructive To be. Current Tretinoin has also been used to reduce the thickness of papules.