What is acrokeratoelastoidosis?

What is acrokeratoelastoidosis?

Acrokeratoelastoidosis (AKE) is a rare genodermatosis characterized by small, firm papules or plaques on the sides of the hands and feet. These nodules may result from an abnormality in the secretion or excretion of elastic material by fibroblasts in the dermis.

What does Keratoderma mean?

Keratoderma is a heterogeneous group of disorders of keratinization defined by the presence of focal or generalized thickening of the skin of the palms and/or soles.

What is focal hyperkeratosis?

Focal acral hyperkeratosis (FAH) is a rare and benign cutaneous disorder characterized by clusters of discrete, small, firm, hyperkeratotic papules along the borders of the hands and feet. The exact etiology and pathogenesis are not known.

Is keratoderma curable?

Inherited palmoplantar keratodermas are not curable but symptoms can be controlled. The aim of treatment is to reduce the thickness of the skin and to soften the skin.

How do you get rid of keratoderma?


  1. Saltwater soaks.
  2. Emollients.
  3. Paring (cutting away layers of skin)
  4. Topical keratolytics (useful for people with limited keratoderma)
  5. Topical retinoids (this is often limited by skin irritation)
  6. Systemic retinoids (acitretin)
  7. Topical vitamin D ointment (calcipotriol)

How is Palmoplantar Keratoderma treated?

Where does hyperkeratosis occur?

Hyperkeratosis is a thickening of the outer layer of the skin. This outer layer contains a tough, protective protein called keratin. This skin thickening is often part of the skin’s normal protection against rubbing, pressure and other forms of local irritation. It causes calluses and corns on hands and feet.

How do you get rid of hyperkeratosis?

Warts and actinic keratosis may be removed with cryosurgery or laser therapy. Topical agents used in the treatment of hyperkeratosis may include: Salicylic acid to break up the keratin, causing the thick skin to soften and be shed, thus reducing the thickness of the skin.

Where can I find information about acrokeratoelastoidosis of Costa?

PubMed is a searchable database of medical literature and lists journal articles that discuss Acrokeratoelastoidosis of Costa. Click on the link to view a sample search on this topic. Questions sent to GARD may be posted here if the information could be helpful to others.

Which physical findings are characteristic of acrokeratoelastoidosis?

It characteristically presents with flesh-colored papules on the lateral areas of the palms, soles, and dorsum of hands. Acrokeratoelastoidosis is a type of marginal keratoderma that principally affects the lateral portion of the palmoplantar regions [Figures 1 (A) and (B)].

What are some other names for acrokeratosis?

Symptoms Medical Terms Other Names Learn More: HPO ID Acrokeratosis 0200016 Hyperkeratosis 0000962 Papule 0200034