Poikiloderma-like cutaneous amyloidosis
Alerts and Notices
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
ICD10CM:E85.9 – Amyloidosis, unspecified
SNOMEDCT:
237872005 – Poikilodermal cutaneous amyloid
Look For
Subscription Required
Diagnostic Pearls
Subscription Required
Differential Diagnosis & Pitfalls
Cutaneous PCA:- Amyloidosis cutis dyschromica – Usually manifests more generalized pigmentary change without poikiloderma. This entity will not feature lichenoid papules or bullous lesions.
- Poikilodermatous cutaneous T-cell lymphoma – Differentiation between this entity and PCA can be made with histopathology.
- Confluent and reticulated papillomatosis – Lesions usually first appear on the central chest or back, in contrast to lesions of PCA that favor the extremities.
- Dowling-Degos disease – Reticular hyperpigmentation in this condition is generally in a flexural distribution.
- Rothmund-Thomson syndrome (poikiloderma congenitale) – Photosensitivity, poikiloderma, and sparse hair are accompanied by cataracts, hypogonadism, short stature, and other skeletal abnormalities. Disease onset is generally between a few months and 2 years after birth.
- Dyskeratosis congenita – This condition also includes nail dystrophy, leukoplakia, and pancytopenia.
- Dyschromatosis universalis hereditaria – This can be differentiated histopathologically, as amyloid deposits would not be seen.
Best Tests
Subscription Required
Management Pearls
Subscription Required
Therapy
Subscription Required
References
Subscription Required
Last Reviewed:04/19/2020
Last Updated:05/31/2020
Last Updated:05/31/2020
Poikiloderma-like cutaneous amyloidosis