Indian Journal of Dermatology
  Publication of IADVL, WB
  Official organ of AADV
Indexed with Science Citation Index (E) , Web of Science and PubMed
Users online: 4573  
Home About  Editorial Board  Current Issue Archives Online Early Coming Soon Guidelines Subscriptions  e-Alerts    Login  
    Small font sizeDefault font sizeIncrease font size Print this page Email this page

Table of Contents 
Year : 2021  |  Volume : 66  |  Issue : 4  |  Page : 434-437
Successful Treatment of Poikiloderma-Like Cutaneous Amyloidosis: A Case Report and Literature Review

1 Department of Dermatology, Shin Kong Wu Ho-Su Memorial Hospital, Taipei, Taiwan
2 Department of Dermatology, Shin Kong Wu Ho-Su Memorial Hospital; Department of Dermatology, Kang-Ning General Hospital, Taipei, Taiwan

Date of Web Publication17-Sep-2021

Correspondence Address:
Gong-Yau Chu
Department of Dermatology, Shin Kong Wu Ho-Su Memorial Hospital; Department of Dermatology, Kang-Ning General Hospital, Taipei
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijd.IJD_764_19

Rights and Permissions

How to cite this article:
Lin YL, Chu GY. Successful Treatment of Poikiloderma-Like Cutaneous Amyloidosis: A Case Report and Literature Review. Indian J Dermatol 2021;66:434-7

How to cite this URL:
Lin YL, Chu GY. Successful Treatment of Poikiloderma-Like Cutaneous Amyloidosis: A Case Report and Literature Review. Indian J Dermatol [serial online] 2021 [cited 2022 May 26];66:434-7. Available from:


An 80-year-old hypertensive Taiwanese male presented to us with chronic scalp pruritus for one year without receiving any treatment in the past. The family history was unremarkable. Physical examination revealed a bald scalp with overlying multiple hyperkeratotic and scaly papules and plaques in the inflamed background. Other findings included hyperpigmentation, hypopigmentation, telangiectasia, and epidermal atrophy [Figure 1]. He did not have palmoplantar keratoses, bullous formation, or photosensitivity, and he was of normal stature. In addition, no specific cutaneous lesions were noted in other parts of the body.
Figure 1: Multiple hyperkeratotic and scaly papules and plaques on the scalp

Click here to view

The skin biopsy revealed hyperkeratosis and focal atrophy of the epidermis. Pale eosinophilic, amorphous, and fissured materials were also detected in the papillary dermis [Figure 2]. The special stain of Congo red was positive [Figure 3], indicating amyloid deposition in the papillary dermis. Although we suggested further investigations to rule out systemic amyloidosis, he declined our advice and denied any discomfort. The review of his past six-month medical examinations was unremarkable. Based on the clinical presentation and histological findings, we made the diagnosis of poikiloderma-like primary cutaneous amyloidosis in the scalp. Accordingly, the patient was treated with potent topical steroid (0.05% w/w clobetasol propionate) and cryotherapy. After a three-month treatment, scaly plaques and papules markedly flattened, and the inflamed background of the scalp gradually subsided. In addition, both the pruritus and the mottled skin improved with only some hyperpigmented and hypopigmented macules remaining [Figure 4]. Remarkably, we observed regrowth of his black hair along with the improvement of disease.
Figure 2: Hyperkeratosis and focal atrophy of the epidermis, and pale eosinophilic, amorphous and fissured materials in the papillary dermis. (HE, × 100)

Click here to view
Figure 3: Special stain of Congo red exhibited a dull brick red coloration of amyloid deposition in the papillary dermis (Congo red, ×100)

Click here to view
Figure 4: After a three-month treatment, hyperkeratotic and scaly plaques and papules flattened and the inflamed background of the scalp gradually subsided

Click here to view

PCA was first described in 1936 and classified as a rare variant of cutaneous amyloidosis in 1959.[1] There are two clinical types of PCA: the ordinary type and PCA syndrome.[2] The main features of ordinary PCA are poikilodermatous skin manifestations, lichenoid papules, and late-onset blister formation, whereas PCA syndrome is usually early-onset, characterized by the presence of symptoms of ordinary PCA together with light sensitivity, short stature, and moderate palmoplantar keratosis.[2] Age at onset is critical for distinguishing PCA syndrome from ordinary PCA.[1]

Only few case reports of ordinary PCA and PCA syndrome have been published. Lesions are found on the face, external ear, neck, trunk, and extremities [Table 1].[1],[2],[3],[4],[5],[6],[7],[8] The lesions primarily involved the trunk and limbs, followed by the face. Most patients complained of chronic pruritus. Comparing with the reported cases in [Table 1], our case had localized involvement of the vertex scalp; the exclusive distribution of the lesions has never been reported in the literature. In addition, the case reports were all Asian, except one patient was not mentioned in the article.
Table 1: Summary of PCA and PCA syndrome

Click here to view

There is no single effective therapy for primary cutaneous amyloidosis. Treatment includes corticosteroids, dimethylsulfoxide, calcineurin inhibitors, acitretin, cyclophosphamide, phototherapy, dermabrasion, hydrocolloid dressing, and pulsed-dye laser therapy.[5],[6],[7] Our patient preferred topical therapy first and gained a satisfactory result.

In conclusion, we report the rare variant and unique distribution of poikiloderma-like cutaneous amyloidosis. Hence, dermatologists should be cautious about the clinical and pathological presentation to correctly diagnose patients presenting with poikiloderma-like lesions.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Ho MH, Chong LY. Poikiloderma-like cutaneous amyloidosis in an ethnic Chinese girl. J Dermatol 1998;25:730-4.  Back to cited text no. 1
Ogino A, Tanaka S. Poikiloderma-like cutaneous amyloidosis. Report of a case and review of the literature. Dermatologica 1977;155:301-9.  Back to cited text no. 2
Wang X, Wen S, Sun J. Poikiloderma-like cutaneous amyloidosis accompanied by generalized milia in a Chinese man. J Dermatol 2017;44:476-7.  Back to cited text no. 3
Wu J, Qian Y, Liu Y, Chen J, Yin X, Long FQ. Poikiloderma-like cutaneous amyloidosis associated with papillary thyroid carcinoma. J Dermatol 2018;45:241-3.  Back to cited text no. 4
Heng JK, Ho SA, Tan KB. Poikiloderma-like cutaneous amyloidosis–A rare presentation of primary localized cutaneous amyloidosis. Dermatol Online J 2016;22:3.  Back to cited text no. 5
Chandran NS, Goh BK, Lee SS, Goh CL. Case of primary localized cutaneous amyloidosis with protean clinical manifestations: Lichen, poikiloderma-like, dyschromic and bullous variants. J Dermatol 2011;38:1066-71.  Back to cited text no. 6
Zeng YP, Jin HZ, Fang K. Poikiloderma-like primary cutaneous amyloidosis confined to the calves in a Chinese man. J Dtsch Dermatol Ges 2012;10:663-4.  Back to cited text no. 7
Ma H, Su X, Zhu G, Yin S, Lu C, Lai W. Primary localized cutaneous amyloidosis with lichen and poikiloderma-like lesions and an excellent response to systemic acitretin. An Bras Dermatol 2016;91:661-3.  Back to cited text no. 8


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

  [Table 1]


Print this article  Email this article
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Article in PDF (1,468 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  

    Article Figures
    Article Tables

 Article Access Statistics
    PDF Downloaded26    
    Comments [Add]    

Recommend this journal