Preferred Label : Lipoid proteinosis;
ICD-11 definition : Lipoid proteinosis (LiP), also known as 'hyalinosis cutis et mucosae' or 'Urbach-Wiethe
disease' is an autosomal recessive disorder that presents in early infancy with hoarseness,
followed by pox-like and acneform scars, along with infiltration and thickening of
the skin and certain mucous membranes due to deposition of hyaline-like material and
disruption/reduplication of the basement membrane around blood vessels and at the
dermal-epidermal junction.;
ICD-11 synonym : Urbach lipoid proteinosis; Urbach-Wiethe disease;
Origin ID : 326368380;
Automatic exact mappings (from CISMeF team)
Lipoid proteinosis (LiP), also known as 'hyalinosis cutis et mucosae' or 'Urbach-Wiethe
disease' is an autosomal recessive disorder that presents in early infancy with hoarseness,
followed by pox-like and acneform scars, along with infiltration and thickening of
the skin and certain mucous membranes due to deposition of hyaline-like material and
disruption/reduplication of the basement membrane around blood vessels and at the
dermal-epidermal junction.
