Nodular cutaneous amyloidosis

Orpha code: 137810OMIM code:

Definition

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).

Disease data
Classification

Disease

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

No additional description.

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