Poikiloderma‐like Cutaneous Amyloidosis in an Ethnic Chinese Girl

Primary cutaneous amyloidosis is the deposition of amyloid in the skin without involvement of internal organs. It is easily diagnosed when presented in its typical manifestation. Atypical or rare clinical presentations can pose diagnostic difficulties. Poikiloderma‐like cutaneous amyloidosis (PCA),...

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Bibliographic Details
Published in:Journal of dermatology 1998-11, Vol.25 (11), p.730-734
Main Authors: Ho, Man Hon, Chong, L.Y.
Format: Article
Language:English
Subjects:
Adult
amyloidosis
Amyloidosis - diagnosis
Amyloidosis - pathology
Biopsy, Needle
Diagnosis, Differential
Female
Humans
poikiloderma
Rothmund-Thomson Syndrome - diagnosis
Rothmund-Thomson Syndrome - pathology
Skin Diseases - diagnosis
Skin Diseases - pathology
syndrome
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Summary:Primary cutaneous amyloidosis is the deposition of amyloid in the skin without involvement of internal organs. It is easily diagnosed when presented in its typical manifestation. Atypical or rare clinical presentations can pose diagnostic difficulties. Poikiloderma‐like cutaneous amyloidosis (PCA), a rare variant of primary cutaneous amyloidosis, was first reported in the literature in 1936 (1). It is characterised by: 1) poikilodermatous skin lesions; 2) lichenoid papules; 3) cutaneous amyloid deposit in the pigmented and lichenoid lesions; 4) light sensitivity; 5) short stature; and 6) other features such as blister formation or palmoplantar keratosis. Ogino coined the term PCA syndrome when these unusual features present early in life (2). We report a 26‐year‐old Chinese woman who presented with poikilodermatous skin lesions and was misdiagnosed as poikiloderma atrophica vasculare (PAV) on the basis of clinical appearance without any histological proof. The diagnosis of PCA was made after skin biopsy which showed amyloid deposits in the skin. This condition can easily be confused with other true poikiloderma skin diseases. Histology is important in confirming the diagnosis.
ISSN:0385-2407
1346-8138
DOI:10.1111/j.1346-8138.1998.tb02492.x