Meleda disease, also called mal de Meleda or Mljet disease, is an extremely rare congenital skin disorder inherited in an autosomal recessive pattern. Dry, thick patches of skin form on the soles of the feet and the palms of the hands, a state known as palmoplantar hyperkeratosis. It can usually be recognized soon after birth. The hands and feet are normally affected first, but the disease can spread to other parts of the body, and the skin may thicken and turn red. The source lists many alternative names, including acral keratoderma and palmoplantar keratoderma of the Norrbotten type. There is no cure and treatment is limited, though acitretin can be used in severe cases.
Facts
Classification
ICD-10 ChapterXVII: Congenital Malformations, Deformations and Chromosomal Abnormalities 2 Communicable or Non-communicable Transmission Route Causative Agent
Causative Agent (category) Prevalence Band Sources
1. Meleda disease (Wikipedia)
Lead section
extremely rare autosomal recessive congenital skin disorder
Lead
Meleda disease (MDM) or "mal de Meleda", also called Mljet disease, keratosis palmoplantaris and transgradiens of Siemens, (also known as "acral keratoderma", "mutilating palmoplantar keratoderma of the Gamborg-Nielsen type", "palmoplantar ectodermal dysplasia type VIII", and "palmoplantar keratoderma of the Norrbotten type") is an extremely rare autosomal recessive congenital skin disorder in which dry, thick patches of skin develop
View the Source2. Orphadata (Orphanet): Orphadata cross-references for ORPHA:87503 Meleda disease
Orphadata (Orphanet)ICD-10 cross-referenceQuote, ICD-10 cross-reference
"Reference": "Q82.8", "Source": "ICD-10"
View the Source 3. Orphadata (Orphanet): Orphadata epidemiology for ORPHA:87503 Mal de Meleda
Orphadata (Orphanet)Prevalence, WorldwideQuote, Prevalence, Worldwide
"PrevalenceClass": "1-9 / 100 000", "PrevalenceGeographic": "Worldwide"
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