Meleda disease (MDM) or "mal de Meleda", also called Mljet disease, keratosis palmoplantaris and transgradiens of Siemens, (also known as "acral keratoderma", and "palmoplantar keratoderma of the Norrbotten type") is an extremely rare autosomal recessive congenital skin disorder in which dry, thick patches of skin develop on the soles of the hands and feet, a condition known as palmoplantar hyperkeratosis. Meleda Disease is a skin condition which usually can be identified not long after birth. This is a genetic condition but it is very rare. The hands and feet usually are the first to show signs of the disease but the disease can advance to other parts of the body. Signs of the disease include thickening of the skin, on hands and soles of feet, which can turn red in color. There currently is no cure and treatment is limited, but Acitretin can be used in severe cases.
Signs and symptoms
Skin on the palms of hands and soles of feet have dry, thick patches which progress slowly. The skin that is affected may look red in color and then start becoming abnormally thick and scaly.
Other symptoms can include excessive sweating due to the thick skin affecting sweat glands on the skin; this excessive sweating can cause a person to have bad odor.
Genetic
MDM is most common on the Dalmatian island of Mljet (), thought to be because of a founder effect. It is of autosomal recessive inheritance. It may be caused by a mutation on the SLURP1 gene, located on chromosome 8. The SLURP1 gene makes a protein called SLURP-1, and this protein is located in cell (biology)s of the skin. The protein SLURP-1 helps with cell death regulation and help mediate inflammation that is occurring, A gene mutation would be caused by the chromosome 8qter, but was taken off of the market in 1998 in America due to toxic effects and the increased risk of birth defects. Aromatic Retinoid Etretinate is still sold in Japan under the name Tigason. In America, Etretinate was replaced by Acitretin, and is only used in severe cases due to the severe side effects. If taking Acitretin it is advised to not donate blood or get pregnant for at least 3 years after taking the drug.
Topical Lotion
Topical lotions can help keep the skin moisturized, and help reduce flaking of the skin.