Meleda Disease

Meleda Disease

Article
Skin Care
Diseases & Conditions
+1
Contributed byKrish Tangella MD, MBANov 17, 2018

What are the other Names for this Condition? (Also known as/Synonyms)

  • Keratosis Palmoplantaris Transgradiens of Siemens
  • Mal de Meleda 

What is Meleda Disease? (Definition/Background Information)

  • Meleda Disease is a genetic disorder characterized by red, dry, and thickened skin that starts-off on the soles of the hands and/or feet, with a capacity to progress to different areas of the body throughout an individual’s lifetime
  • The risk factors include a family history of the disorder and having parents that are closely related by blood. Meleda Disease is an autosomal recessive genetic disorder caused by a defective ARS gene. It presents symptoms at birth or shortly after birth
  • The other signs and symptoms of Meleda Disease may include the skin turning yellowish after thickening, redness of the mouth, nail and hair abnormalities, excessive sweating, webbed or short fingers or toes, and cracked/grooved tongue
  • A diagnosis of Meleda Disease includes a careful physical examination, searching for characteristic findings (such as redness of the palms/soles), and a thorough evaluation of a patient’s medical history
  • Treatments include surgery, drug therapy, lotions and other topical medications, and genetic counseling for affected individuals and their family members. Chronic fungal infections may have to be treated using antifungal medications
  • The prognosis for Meleda Disease is generally very good; while quality of life may be decreased, the affected individuals can manage the severity of the symptoms through appropriate treatment

Who gets Meleda Disease? (Age and Sex Distribution)

  • Meleda Disease displays symptoms at birth (congenital) or shortly after birth
  • Males and females can both be affected
  • All races and ethnic groups can be affected and no specific preference is noted

What are the Risk Factors for Meleda Disease? (Predisposing Factors)

Predisposing factors for Meleda Disease may include:

  • Having a parent with the disorder; as it can be inherited genetically
  • Parents who are closely-related by blood have an increased risk for a child being born with Meleda Disease

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others. 

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Meleda Disease? (Etiology)

Meleda Disease is an autosomal recessive inherited genetic disorder.

  • Both parents must pass on the defective gene for the child to be affected
  • The defective gene is known as the ARS gene on the long arm of chromosome 8
  • This gene codes for the protein SLURP-1, which is believed to aid in cell adhesion and signaling

Autosomal recessive: Autosomal recessive conditions are traits or disorders that occur when two copies of an abnormal gene have been inherited on a non-sex chromosome. If both parents have an autosomal recessive condition, there is a 100% likelihood of passing on the mutated genes to their children. If, however, only one mutant copy of the gene is inherited, the individual will be a carrier of the condition, but will not be present with any symptoms. Children, born to two carriers, have a 25% chance of being homozygous dominant (unaffected), a 50% chance of being heterozygous (carrier), and a 25% chance of being homozygous recessive (affected). 

What are the Signs and Symptoms of Meleda Disease?

The signs and symptoms of Meleda Disease may include:

  • Redness of the palms of the hands and soles of the feet which is either apparent at birth (congenital) or seen shortly after birth
  • The affected skin area thickens, hardens, and becomes yellow-brown in color; the affected skin area may present scale-like features
  • As the child grows older this may spread to the arms, legs, and even to the chest and abdomen
  • The affected skin areas can be very painful

Other symptoms may include:

  • Small, raised lesions on affected areas of the skin
  • Redness around the mouth
  • Abnormalities of the nails, such as increased hardness or thickness, spoon-shaped or hook-shaped nails
  • Excessive hair growth on the hands and/or feet
  • Excessive sweating and an unpleasant body odor
  • Webbed fingers or toes; unusually short fingers or toes
  • Cracked or grooved tongue

How is Meleda Disease Diagnosed?

A diagnosis of Meleda Disease may include the following:

  • A careful physical examination and evaluation of medical history
  • An examination of characteristic findings (such as redness of palms and/or soles of feet following birth of the child)
  • Other tests may be undertaken based on the healthcare provider’s assessment

What are the possible Complications of Meleda Disease?

The possible complications associated with Meleda Disease include:

  • Recurrent fungal infections because of the thickened skin
  • Severe pain can cause prolonged discomfort in children
  • Decreased quality of life

How is Meleda Disease Treated?

The treatment for Meleda Disease is planned through a coordinated effort of many physicians in various specialties. The treatments may vary depending on the affected individual’s condition and may be specifically-suited to each individual.

The following treatment measures may be considered:

  • Surgery
  • Drug therapy
  • Lotions and other topical medications

How can Meleda Disease be Prevented?

  • Currently, there are no specific methods or guidelines to prevent Meleda Disease, since it is a genetic condition
  • Genetic testing of the expecting parents (and related family members) and prenatal diagnosis (molecular testing of the fetus during pregnancy) may help in understanding the risks better during pregnancy
  • If there is a family history of the condition, then genetic counseling will help assess risks, before planning for a child
  • Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic disorders such as Meleda Disease

Regular medical screening at periodic intervals with tests and physical examinations are recommended.

What is the Prognosis of Meleda Disease? (Outcomes/Resolutions)

  • The prognosis for Meleda Disease is generally very good with appropriate treatment
  • While the quality of life may be decreased, most individuals can manage with the disorder through treatment. Also, physical and mental growth and development remains unaffected

Additional and Relevant Useful Information for Meleda Disease:

The first report of Meleda Disease came from the island of Meleda in Yugoslavia.

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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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