Poikiloderma-like cutaneous amyloidosis
Synopsis

There are 2 forms of PCA. One type is restricted to cutaneous involvement. The second form is known as PCA syndrome, which includes PCA, short stature, photosensitivity, and palmoplantar keratosis. While individuals with PCA syndrome generally have evidence of skin involvement in childhood, the skin-limited forms may appear later in life. While most reported cases are sporadic, familial cases have been described.
Codes
E85.9 – Amyloidosis, unspecified
SNOMEDCT:
237872005 – Poikilodermal cutaneous amyloid
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Last Updated:05/31/2020