DEVELOPMENT PIPELINE

Palmoplantar Keratodermas

Palmoplantar Keratodermas

Severe Rare Skin Diseases

Severe Rare Skin Diseases

The Palmoplantar Keratodermas (PPKs)

Topical cream for the treatment of Palmoplantar Keratodermas (PPKs), a group of rare skin conditions with great unmet need.
Group of rare skin conditions that result from various mutations in several genes. PPKs are characterized by thickening of the skin on the palms and soles leading to great discomfort, itch and pain. The PPKs include diseases such as Punctuate PPK, Pachyonychia Congenita (PC) and Mal de Meleda, all of which have no approved treatment. Kamari’s TRPV3 inhibitors, if approved, may be promising treatment for these conditions.
Kamari pharma is collaborating with PC project to raise awareness for PC and other PPK related conditions. The PC project is a dedicated organization to find effective treatments and a cure for PC. www.pachyonychia.org

photograph courtesy of the PC Project

Placebo

Placebo

Response rate

skin normalization according to histology

Treated with KM-001 1%

Treated with KM-001 1%

Response rate

skin normalization according to histology

Effect of Tested Treatments on Skin

Morphology Normalization of DS-Nh mice

Advanced oral treatment for severe Keratoderma and Ichthyosis

Olmsted syndrome and severe Palmoplantar Keratoderma
Olmsted Syndrome is an ultra rare genetic disorder, characterized by the development of severe and sometimes mutilating hyperkeratotic plaques, diffuse baldness (alopecia), extreme itch (pruritus), and debilitating pain.
‘Gain-of-function’ mutations in the TRPV3 gene lead to a constant activation of the TRPV3 channel, thus overflow of Ca²⁺ skin cells.

Ichthyosis
The ichthyoses are disorders of keratinization or cornification. They are characterized by generalized scaling of the skin in varying severity with or without erythroderma. Most ichthyols disease forms are inherited but acquired forms can develop in the setting of certain cancers, autoimmune or infectious disease, or nutritional deficiency.

Harlequin Ichthyosis
One of the most severe of the Ichthyoses is Harlequin Ichthyosis (HI). Presenting at birth, infants born with this ultra rare condition are covered in thick plate-like scales of skin. HI is often fatal, and patients die due to complications of sepsis, dehydration, or respiratory failure. There are currently no treatments available for those who suffer from HI, other than supportive care.