ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferencesView all Images (2)
Poikiloderma-like cutaneous amyloidosis
Other Resources UpToDate PubMed

Poikiloderma-like cutaneous amyloidosis

Contributors: Jeffrey M. Cohen MD, Susan Burgin MD
Other Resources UpToDate PubMed

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.

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.
PCA syndrome:
  • 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:04/20/2020
Last Updated:06/01/2020
Copyright © 2021 VisualDx®. All rights reserved.
Poikiloderma-like cutaneous amyloidosis
Poikiloderma-like cutaneous amyloidosis (Cutaneous Presentation)
Copyright © 2021 VisualDx®. All rights reserved.