Meleda Disease
Definition:

Its peculiar name is derived from the Dalmatian island of Melda because its founder was said to have been afflicted with the disease. Extremely rare, meleda disease is an inherited disorder of the skin characterized by thick, dry patches of skin which appear on the soles of both hands and feet (palmoplantar hyperkeratosis).


Diagnosis:

In diagnosing meleda diseases, care must be given to differentiate it from other hyperkeratotic conditions with similar symptoms such as psoriasis, lichen planus and icthyosis.


Treatment:

Drug therapy remains the best choice for treatment. The use of oral retinoids has been proven to be effective in both children and adults although caution is advised for women patients who plan to get pregnant. The drug also presents side-effects such as mucosal dryness, epistaxis and cheilitis


Symptoms and Signs:

Symptoms include skin that is abnormally red in color and develop symmetrical cornification which means that it becomes scaly and really thick. In children who have the disease, other symptoms include nail deformations, hyperhidrosis and the development of lichenoid plaques as a result of excessive sweating.


Causes:

The cause for this disease is genetics; classified as being autosomal recessive inheritance, offspring can be carriers or who later develop the disease. Studies have shown that the mutation may occur on the SLURP gene.


:

meledadisease



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