Poikiloderma‐like Cutaneous Amyloidosis in an Ethnic Chinese Girl

Abstract

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.

A clinico-epidemiological study of macular amyloidosis from North India

“…[310] Unusual presentations of MA, such as nevoid like hyperpigmentation, widespread diffuse pigmentation, poikiloderma like, incontinentia pigment like and linear MA have frequently been reported in the literature. [2230–33] A rippled pattern of pigmentation was present in all of our patients without any special pattern or distribution.…”

Cutaneous Amyloidosis: A Concept Review

“…Patients with this syndrome present sensitivity to light, short stature, and other cutaneous acompanying alterations such as blisters or palmoplantar keratosis. 221,222 There is also a familial form of localized cutaneous amyloidosis 223 inherited in an autosomal dominant form. 224,225 Although it is most common in Asian and South American populations, it has also been described in Europe.…”

Unusual Skin Manifestation of Cutaneous Amyloidosis

“…Poikiloderma-like cutaneous amyloidosis (PCA) was first reported by Marchionini and John in 1936 [4]. The major features of PCA are poikilodermatous skin lesions, lichenoid papules and histological evidence of amyloid deposit with occasional light sensitivity, short stature, palmoplantar hyperkeratosis and blister formation [5]. Ogino and Tanaka [6]introduced the term ‘poikiloderma-like cutaneous amyloidosis syndrome’ (PCA syndrome) in 1977.…”