How Do You Spell MAL DE MELEDA?

1. Mal de Meleda is a rare and inherited genodermatosis characterized by thickening and scaling of the skin on the palms of the hands and soles of the feet. This condition is considered a type of palmoplantar keratoderma (PPK), a group of disorders that cause abnormal thickening of the skin on the palms and soles.

   Individuals affected by mal de Meleda usually experience the onset of symptoms during early childhood. The thickening and scaling of the skin typically begins on the palms and soles and may gradually spread to other parts of the body. The skin abnormalities can cause discomfort, pain, and impair the individual's ability to perform tasks that require manual dexterity. In severe cases, the thickened skin may limit the movement of the fingers and toes, leading to physical disabilities.

   Mal de Meleda is caused by mutations in the gene known as SLURP1 (secreted mammalian Ly-6/uPAR related protein 1). This gene provides instructions for producing a protein that is involved in the normal development and maintenance of the skin. Mutations in SLURP1 lead to the production of an abnormal protein or a complete lack of the protein, which disrupts the normal functioning of the skin cells.

   While there is currently no cure for mal de Meleda, treatment is primarily focused on managing and alleviating the symptoms. This may include the use of emollients, keratolytic agents, and other topical therapies to soften and remove the thickened skin. In some cases, surgical interventions such as skin grafting or the use of medications like retinoids may be considered to improve the condition of the skin. Genetic counseling may also be recommended for affected individuals and their families to understand the inheritance pattern and

2. Endemic symmetrical keratodermia of the extremities occurring on the island of Meleda off the coast of Dalmatia.

   A practical medical dictionary. By Stedman, Thomas Lathrop. Published 1920.

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