Poikiloderma-like cutaneous amyloidosis
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.
E85.9 – Amyloidosis, unspecified
237872005 – Poikilodermal cutaneous amyloid
Differential Diagnosis & Pitfalls
- – Usually manifests more generalized pigmentary change without poikiloderma. This entity will not feature lichenoid papules or bullous lesions.
- Poikilodermatous – Differentiation between this entity and PCA can be made with histopathology.
- – Lesions usually first appear on the central chest or back, in contrast to lesions of PCA that favor the extremities.
- – Reticular hyperpigmentation in this condition is generally in a flexural distribution.
- (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.
- – This condition also includes nail dystrophy, leukoplakia, and pancytopenia.
- – This can be differentiated histopathologically, as amyloid deposits would not be seen.