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Dermatopathology
Volume 8
Issue 3
10.3390/dermatopathology8030028
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Case Report

Primary Localized Cutaneous Nodular Amyloidosis and Limited Cutaneous Systemic Sclerosis: Additional Cases with Dermatoscopic and Histopathological Correlation of Amyloid Deposition

by
Laura Atzori
1,
Caterina Ferreli
1,
Caterina Matucci-Cerinic
2,*,
Luca Pilloni
3 and
Franco Rongioletti
1,4
1
Dermatology Clinic, Department Medical Sciences and Public Health, University of Cagliari, 09124 Cagliari, Italy
2
Clinica Pediatrica e Reumatologia, IRCCS Istituto Giannina Gaslini, DINOGMI, University of Genova, 16147 Genova, Italy
3
Pathology Unit, Department Medical Sciences and Public Health, University of Cagliari, 09124 Cagliari, Italy
4
Dermatology Unit, Vita-Salute University San Raffaele, 20132 Milan, Italy
*
Author to whom correspondence should be addressed.
Dermatopathology 2021, 8(3), 229-235; https://doi.org/10.3390/dermatopathology8030028
Submission received: 3 May 2021 / Revised: 1 June 2021 / Accepted: 22 June 2021 / Published: 2 July 2021
(This article belongs to the Special Issue In Memory of Raffaele Gianotti)
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Abstract

Primary localized cutaneous nodular amyloidosis (PLCNA) is a rare condition due to the plasma cell proliferation and skin deposition of immunoglobulin light chains, without systemic amyloidosis or hematological dyscrasias. The association with autoimmune connective tissue diseases has been reported, especially with Sjogren’s syndrome, and in a few cases with systemic sclerosis. Herein, we describe three cases of PLCNA occurring in women with a diagnosis of limited cutaneous systemic sclerosis and review the literature on the topic to highlight a stereotypical presentation. Moreover, we support the usefulness of dermoscopy, characterized by a yellow–orange waxy pattern surrounded by telangiectasias, for a rapid and non-invasive diagnostic assessment. Thus, when asymptomatic nodules occur on lower limbs of women affected with limited systemic sclerosis, and dermoscopy identifies yellow–orange blotches, a diagnosis of PLCNA can be considered and further confirmed by histopathology. Monitoring for systemic amyloidosis development is advisable, although the risk of progression is considered very low.
Keywords: primary localized cutaneous nodular amyloidosis; dermoscopy; limited cutaneous systemic sclerosis; connective tissue disease primary localized cutaneous nodular amyloidosis; dermoscopy; limited cutaneous systemic sclerosis; connective tissue disease

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MDPI and ACS Style

Atzori, L.; Ferreli, C.; Matucci-Cerinic, C.; Pilloni, L.; Rongioletti, F. Primary Localized Cutaneous Nodular Amyloidosis and Limited Cutaneous Systemic Sclerosis: Additional Cases with Dermatoscopic and Histopathological Correlation of Amyloid Deposition. Dermatopathology 2021, 8, 229-235. https://doi.org/10.3390/dermatopathology8030028

AMA Style

Atzori L, Ferreli C, Matucci-Cerinic C, Pilloni L, Rongioletti F. Primary Localized Cutaneous Nodular Amyloidosis and Limited Cutaneous Systemic Sclerosis: Additional Cases with Dermatoscopic and Histopathological Correlation of Amyloid Deposition. Dermatopathology. 2021; 8(3):229-235. https://doi.org/10.3390/dermatopathology8030028

Chicago/Turabian Style

Atzori, Laura, Caterina Ferreli, Caterina Matucci-Cerinic, Luca Pilloni, and Franco Rongioletti. 2021. "Primary Localized Cutaneous Nodular Amyloidosis and Limited Cutaneous Systemic Sclerosis: Additional Cases with Dermatoscopic and Histopathological Correlation of Amyloid Deposition" Dermatopathology 8, no. 3: 229-235. https://doi.org/10.3390/dermatopathology8030028

APA Style

Atzori, L., Ferreli, C., Matucci-Cerinic, C., Pilloni, L., & Rongioletti, F. (2021). Primary Localized Cutaneous Nodular Amyloidosis and Limited Cutaneous Systemic Sclerosis: Additional Cases with Dermatoscopic and Histopathological Correlation of Amyloid Deposition. Dermatopathology, 8(3), 229-235. https://doi.org/10.3390/dermatopathology8030028

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