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. 2016 Mar-Apr;61(2):216-8.
doi: 10.4103/0019-5154.177767.

Primary Systemic Amyloidosis with Unusual Dermatological Manifestations: A Rare Case Report

Kapil Vyas  1 , Manjaree Morgaonkar  1 , Savera Gupta  1 , Suresh Kumar Jain  1
Affiliations

Primary Systemic Amyloidosis with Unusual Dermatological Manifestations: A Rare Case Report

Kapil Vyas et al. Indian J Dermatol. 2016 Mar-Apr.

Abstract

Amyloidosis is a group of heterogeneous diseases characterized by pathological deposition of proteinaceous substance extracellularly in various tissues. The clinical presentation depends on the site of amyloid deposition, with predominant involvement of mesenchymal elements and cutaneous findings in 30-40% of patients in case of primary systemic amyloidosis. We present a case of idiopathic primary systemic amyloidosis presenting with an unusual finding of nodulo-ulcerative lesion over tongue along with multiple skin-colored nodules, mimicking squamous cell carcinoma of tongue with secondary cutaneous metastasis, as well as lacking the classical presentation of purpura, macroglossia, waxy papules, and plaques.

Keywords: Amyloidosis; nodules; purpura.

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Figures

Figure 1
Figure 1
(a) Cutis verticis gyrata-like appearance of scalp, (b) multiple skin-colored nodules over back
Figure 2
Figure 2
Nodulo-ulcerative lesion on tip and left lateral border of the tongue, also showing a few nodules on lips
Figure 3
Figure 3
Eosinophilic homogenous material in the dermis and peri-adnexal region (H and E, ×400)
Figure 4
Figure 4
Congo red positivity (×400)
See this image and copyright information in PMC

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