Poikiloderma-like cutaneous amyloidosis
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Synopsis
Poikiloderma-like cutaneous amyloidosis (PCA) is an exceedingly rare form of primary cutaneous amyloidosis (a category which also includes lichen, macular, and nodular amyloidosis, primary cutaneous amyloidosis of the auricular concha, and the equally uncommon entity of amyloidosis cutis dyschromica) that presents with poikiloderma, lichenoid papules, and sometimes bullous lesions. The typical distribution is on the extremities; however, cases of truncal involvement have been reported. Pruritus is a common symptom.
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.
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
ICD10CM:
E85.9 – Amyloidosis, unspecified
SNOMEDCT:
237872005 – Poikilodermal cutaneous amyloid
E85.9 – Amyloidosis, unspecified
SNOMEDCT:
237872005 – Poikilodermal cutaneous amyloid
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Last Reviewed:04/19/2020
Last Updated:05/31/2020
Last Updated:05/31/2020