Guzkowata amyloidoza skórna

Kod Orpha: 137810Kod OMIM:

Definicja

Primary localized cutaneous nodular amyloidosis (PLCNA) is the most rare form of primary cutaneous amyloidosis (see this term), a skin disease characterized by the accumulation of amyloid deposits in the dermis, characterized clinically by yellowish waxy crusted nodules and papules on the face, lower extremities, trunk, scalp, and genitalia and histologically by the localized deposition of immunoglobulin-derived amyloid in the papillary dermis and subcutis. PLCNA can be associated with connective tissue disorders such as Sjögren’s syndrome and CREST syndrome (see these terms).

Dane
Klasyfikacja

Choroba

Synonimy
PLCNA
Pierwotna zlokalizowana guzkowata amyloidoza skórna
plCNA
Primary localized cutaneous nodular amyloidosis
Kod ORPHA
137810
Kod OMIM
-
Kod ICD10
L99.0*
Kod ICD11
5D00.0

Brak opisu rozszerzonego dla tej choroby. Opracowanie w toku.

Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl