Palmoplantar Keratoderma (PPK)  Market

Palmoplantar Keratoderma (PPK)  Market

Introduction

Palmoplantar keratodermas (PPK) is a group of illnesses characterized by the thickening of the skin on the

palms and soles of affected individuals' feet. PPK can be classified into different categories such as

hereditary forms with only skin complications, hereditary syndromes with PPK as an associated feature,

and acquired forms. Hereditary PPK is the most common form of PPK which is generally diagnosed in the

affected patients. Hereditary forms can be limited to the hands and feet, or they might be linked to a more

widespread skin condition. Genetic mutations produce inherited PPKs, which result in keratin (a skin

protein) abnormalities. Autosomal dominant or autosomal recessive inheritance can occur depending on

the genetic reason. Acquired PPKs, on the other hand, might occur as a result of changes in a person's

health or surroundings.

Besides this, PPK can be differentiated from one another depending upon the epidermal involvement

(diffuse, focal and punctuate). The palmoplantar surface is uniformly involved in diffuse PPK while, focal

PPK consists of localized areas of hyperkeratosis majorly present on sites of pressure point and recurrent

friction. The punctate PPK presents multiple small, nodules or hyperkeratotic papules on the soles and

palms

Hereditary palmoplantar keratodermas (PPK) are classified into:

4.1.1 Diffuse Hereditary PPK:

• Epidermolytic PPK (Vorner PPK): Also known as diffuse Vorner disease. In Northern Ireland, it

has an estimated prevalence of approximately 4.4 cases per 100,000 population.

• Nonepidermolytic PPK (Unna-Thost PPK and Bothnian-type PPK): It is another common type

of hereditary PPK with a prevalence rate of 0.3-0.55% in Northern Sweden (near the Gulf of

Bothnia)

• Mal de Meleda: It is is an autosomal recessive disease. The prevalence is 1 case per 100,000

population

• Nagashima-type PPK: Inherited in an autosomal recessive manner.
• Progressive PPK (Greither disease): Inherited in an autosomal dominant fashion

4.1.2 Focal and Striate Hereditary PPK

• Also known as keratosis palmoplantaris nummularis and hereditary painful callosities. Clinical

features consist of nummular keratotic lesions, majorly located on plantar pressure points.

• Striate Palmoplantar Keratodermas is inherited in an autosomal dominant manner. Clinical features

include marked variability in phenotypic expression.

4.1.3 Punctate Hereditary PPK

• Punctate keratosis of the palms and soles (Buschke-Fischer-Brauer type): Synonyms are

punctate PPK type I, keratosis punctata palmaris et plantaris, Buschke-Fischer-Brauer disease.

The disease is passed down through the generations in an autosomal dominant pattern. The

prevalence is 1.17 cases per 100,000 population.

4.2 Causes

Abnormal skin protein (keratin) caused by a gene abnormality results in hereditary keratodermas. This type

of condition may be inherited either by autosomal recessive or an autosomal dominant pattern.

Autosomal dominant keratodermas probably occur in every generation of a family. If a parent is affected

there is a 50% probability for each of the child (1 in 2) to be affected with the disease. While in some cases

mutation arises for the first time in an individual with no family history of the condition, it is termed as de

novo mutation.

Autosomal recessive keratodermas are very unlikely to occur within an affected family. This is because the

child can only contract the disease when an abnormal gene is passed on by both the parents to the child.

People with one affected gene are referred to as “carriers” of the disease and do not have the condition

themselves. When two carriers of this condition have children, each child has a chance of:

• 25% to be affected
• 50% to be unaffected carrier like each parent
• 25% to be unaffected and not a carrier

Acquired PPK usually occur later in life and may be due to many causes, such as drugs, keratoderma

climactericum, chemicals, malnutrition, malignancy, systemic disease, dermatoses, infections and

idiopathies.

4.3 Pathophysiology

The treatment for punctuate PPK is topical retinoids or calcipotriol. For other types, keratolytic agents such

as Systemic retinoids, Tretinoin, and infusion 5-Fluorouracil.

Currently, there is no cure for hereditary palmoplantar keratoses. Acquired PPK patient’s cause should be

treated (infection, toxins, other factors) or eliminated, if possible. In both scenarios, optimized treatment

can lead to a considerable improvement in symptoms. A wide range of Regular baths hydrate and cleanse

areas of keratinization.

• Topical therapy with urea-based ointments is also prescribed to improve the skin’s absorption of

moisture. Besides this Topical vitamin D therapy is another option.

• Systemic therapy along with retinoids (generally acitretin), despite of the side-effects can possibly

lead to marked improvement of palmoplantar hyperkeratosis. Oral retinoids are effective in some

hereditary PPKs such as Papillon-Lefevre syndrome, Mal de Meleda, and erythrokeratodermia

variabilis (a form of ichthyosis that often includes palmoplantar keratoderma). Most hereditary PPKs

require long-term treatment. However, caution is advised if the patient has a blistering form, since

large erosions may occur with oral retinoids therapy.

• In patients with blistering or epidermolytic PPK, retinoid therapy can cause detachment of large

areas of the skin and erosions. Retinoids can cause birth abnormalities, and they can be retained

in adipose tissue for up to 24 months after they've stopped being used.

• In the case of severe type keratoderma, surgery may need to be considered. In a few cases, the

total excision of hyperkeratotic skin is recommended which is further followed by grafts. In addition

to this, Dermabrasion is also important in order to help topical agents to penetrate, and in limited

keratodermas cases, carbon dioxide laser treatment might be beneficial.

Thus, the most commonly used treatments are 10% lactic acid , 5 – 10% salicylic acid, or 10% urea in a

neutral base, topical retinoids, such as tretinoin (0.05% gel and 0.1% cream), and Topical steroids in

conditions where there is an inflammatory component.

Market Size

• 2020: USD 1,098.9 Million
• 2025: USD 1,238.2 Million
• 2030: USD 1,458.8 Million

CAGR (2020– 2030)

• 2.87%

Key Geographies

• United States
• Japan

Key Market Drivers

• Persistent research & development towards understanding the

pathophysiology

• Rise in prevalent cases of chronic patients

Key Market Barriers

• Stringent rules and regulations
• High cost involved in research & development