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Dermoscopy of Primary Localized Cutaneous Nodular Amyloidosis
Authors Pulgarin LM , De Pellegrin A, Stinco G, Errichetti E
Received 9 December 2023
Accepted for publication 6 February 2024
Published 8 February 2024 Volume 2024:17 Pages 395—398
DOI https://doi.org/10.2147/CCID.S451378
Checked for plagiarism Yes
Review by Single anonymous peer review
Peer reviewer comments 2
Editor who approved publication: Dr Jeffrey Weinberg
Laura Manuela Pulgarin,1 Alessandro De Pellegrin,2 Giuseppe Stinco,3 Enzo Errichetti3
1Department of Dermatology, Universidad del Valle, Cali, Colombia; 2Department of Pathology, Udine “S. Maria della Misericordia” University Hospital, Udine, Italy; 3Institute of Dermatology, Department of Medicine, University of Udine, Udine, Italy
Correspondence: Enzo Errichetti, Institute of Dermatology, Department of Medical Area, University of Udine, Piazzale Santa Maria della Misericordia, 15, Udine, 33100, Italy, Tel +39 0432559822, Email [email protected]
Primary localized cutaneous nodular amyloidosis (PLCNA) is a rare skin condition typified by extracellular dermal deposition of amyloid proteins derived from immunoglobulin light chain L protein released by a localized infiltrate of plasma cells. It usually presents as solitary or multiple asymptomatic nodular lesions and its progression to systematic disease is estimated to occur in approximately 7% of the cases,1–6 thus its recognition is of key importance to prompt diagnose any possible extra-cutaneous involvement. In this regard, dermoscopy has been shown to highlight some findings that may support PLCNA recognition, thereby facilitating the differential diagnosis with its clinical mimickers, mainly including cutaneous lymphomas and granulomatous dermatoses.1–6
Here, we present a case of PLCNA with dermoscopic findings, also reviewing existing literature data on this topic and providing a homogeneous terminology according to the standardized dermoscopic criteria for non-neoplastic dermatoses released by the International Dermoscopy Society.7
A 75-year-old female presented with a 1-year history of asymptomatic pink-brown nodules on the posterior part of her left leg (Figure 1a). Medical history included systemic sclerosis and Hashimoto’s thyroiditis. Dermoscopic assessment revealed a diffuse structureless orange area along with linear-curved vessels and few purple (hemorrhagic) globules/focal structureless areas (Figure 1b). Histological examination (Figures 1c and d) showed deposits of pale eosinophilic amorphous substance in the superficial dermis with hemorrhagic infiltrates at the dermo-hypodermal border; Congo red staining was positive (Figure 1e) and a green apple birefringence was evident under polarized light (Figure 1f). Therefore, a diagnosis of PLCNA was made.
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Figure 1 Pink-brown nodules and papules on the left leg (a). Polarized-light dermoscopy shows a diffuse Orange structureless, along with linear-curved vessels and purpuric (hemorrhagic) globules (arrow) (b). Histology shows deposition of pale eosinophilic amorphous substance in the superficial dermis (c) and hemorrhagic infiltrates at the dermo-hypodermal border (d); H&E x100. Positive staining for Congo red (e) and green apple birefringence under polarized light (f); x200. |
According to the foregoing, the most represented dermoscopic feature in analyzed lesions of the present instance turned out to be a diffuse orange structureless area. This is in line with what described in previous seven cases reported in the literature (summarized in Table 1), that constantly showed orange/yellow structureless areas. Such a finding is likely related to the deposits of amyloid protein in the dermis and subcutis (the so-called “mass effect”).8 Additionally, other features reported in prior instances included purple (hemorrhagic) background/dots (42.9% of cases), linear-curved vessels (42.9% of cases), and white dots/lines (71.4% of cases). Of note, in our patients we did not find white structures, probably as a result of the lack of dermal fibrosis, whereas we observed linear-curved vessels and purple (hemorrhagic) areas. Importantly, the detection of purple areas may be a significant clue in favor of PLCNA diagnosis compared to clinical mimickers showing orange color on dermoscopy, such as granulomatous dermatoses and skin lymphomas, that typically do not feature such a finding.8,9 Indeed, the presence of purple (hemorrhagic) structures is likely to be due to the peculiar vascular damage seen in skin amyloidosis with consequent erythrocytes extravasation. Obviously, comparative analyses are needed to confirm such observations.
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Table 1 Previous Dermoscopy Reports on Dermoscopy of Primary Localized Cutaneous Nodular Amyloidosis |
Data Sharing Statement
Data sharing is not applicable to this article as no datasets were generated or analyzed during the current study.
Compliance with Ethics Guidelines
This article is based on previously conducted studies and does not contain any new studies with human participants or animals performed by the authors. The patient in this manuscript has given informed consent to the publication of case details and institutional approval was not required.
Author Contributions
All authors made a significant contribution to the work reported, whether that is in the conception, study design, execution, acquisition of data, analysis and interpretation, or in all these areas; took part in drafting, revising or critically reviewing the article; gave final approval of the version to be published; have agreed on the journal to which the article has been submitted; and agree to be accountable for all aspects of the work.
Funding
No funding or sponsorship was received for this study or publication of this article.
Disclosure
The authors report no conflicts of interest in this work.
References
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2. Rongioletti F, Atzori L, Ferreli C, Pinna A, Aste N, Pau M. A unique dermoscopy pattern of primary cutaneous nodular amyloidosis mimicking a granulomatous disease. J Am Acad Dermatol. 2016;74(1):e9–e10. doi:10.1016/j.jaad.2015.09.026
3. Cheng YJ, Li Y, Lim KH, Oh CC. Dermatoscope of primary localized cutaneous nodular amyloidosis on hallux nail bed. JAAD Case Rep. 2022;27:46–48. doi:10.1016/j.jdcr.2022.07.005
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6. Bellinato F, Rosina P, Sina S, Girolomoni G. Primary nodular localized cutaneous amyloidosis of the scalp associated with systemic lupus erythematosus. Arch Rheumatol. 2021;37:145–147. doi:10.46497/ArchRheumatol.2022.8817
7. Errichetti E, Zalaudek I, Kittler H, et al. Standardization of dermoscopic terminology and basic dermoscopic parameters to evaluate in general dermatology (non-neoplastic dermatoses): an expert consensus on behalf of the International Dermoscopy Society. Br J Dermatol. 2020;182(2):454–467. doi:10.1111/bjd.18125
8. Errichetti E, Stinco G. Dermatoscopy of Granulomatous Disorders. Dermatol Clin. 2018;36(4):369–375. doi:10.1016/j.det.2018.05.004
9. Errichetti E, Geller S, Zalaudek I, et al. Dermatoscopy of nodular/plaque-type primary cutaneous T- and B-cell lymphomas: a retrospective comparative study with pseudolymphomas and tumoral/inflammatory mimickers by the International Dermoscopy Society. J Am Acad Dermatol. 2022;86(4):774–781. doi:10.1016/j.jaad.2021.10.020
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