Indian Journal of Dermatology
: 2021  |  Volume : 66  |  Issue : 4  |  Page : 434--437

Successful Treatment of Poikiloderma-Like Cutaneous Amyloidosis: A Case Report and Literature Review

Yi-Liang Lin1, Gong-Yau Chu2,  
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

Correspondence Address:
Gong-Yau Chu
Department of Dermatology, Shin Kong Wu Ho-Su Memorial Hospital; Department of Dermatology, Kang-Ning General Hospital, Taipei

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;66:434-437

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 Jan 29 ];66:434-437
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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}

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}{Figure 3}{Figure 4}

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}

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.

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Conflicts of interest

There are no conflicts of interest.


1Ho MH, Chong LY. Poikiloderma-like cutaneous amyloidosis in an ethnic Chinese girl. J Dermatol 1998;25:730-4.
2Ogino A, Tanaka S. Poikiloderma-like cutaneous amyloidosis. Report of a case and review of the literature. Dermatologica 1977;155:301-9.
3Wang X, Wen S, Sun J. Poikiloderma-like cutaneous amyloidosis accompanied by generalized milia in a Chinese man. J Dermatol 2017;44:476-7.
4Wu 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.
5Heng JK, Ho SA, Tan KB. Poikiloderma-like cutaneous amyloidosis–A rare presentation of primary localized cutaneous amyloidosis. Dermatol Online J 2016;22:3.
6Chandran 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.
7Zeng 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.
8Ma 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.