10.5826/dpc.1303a147
text/xml
2160-9381
2926
en
Enzo Errichetti
https://creativecommons.org/licenses/by-nc/4.0
Dermatology Practical & Conceptual
Dermoscopic and Cytological Findings in Scleromyxedema 
Text.Serial.Journal
https://dpcj.org/index.php/dpc
Image Letter
2023-07-31
2022-10-19
2023-07-31
2023-08-24
e2023147
Dermatol Pract Concept
Enzo Errichetti
2023/07/31
10.5826/dpc.1303a147
e2023147
Department of Dermatology, University Hospital “Santa Maria della Misericordia”, Udine, Italy
2160-9381
en
e2023147
Lallas A, Errichetti E, Ioannides D. Dermoscopy in General Dermatology. CRC Press, Boca Raton, FL, 2018.
Dermoscopic and Cytological Findings in Scleromyxedema 
https://dpcj.org/index.php/dpc/article/download/2926/2226
                  Dermoscopic and Cytological Findings
                          in Scleromyxedema
                    Tuğba Tehçi1, Elif Burcu Şenyurt2, Murat Durdu3, Enzo Errichetti4


1 Department of Dermatology, University of Health Sciences -Adana Health Practice and Research Center, Adana, Turkey
2 Department of Pathology, University of Health Sciences -Adana Health Practice and Research Center, Adana, Turkey
3 Department of Dermatology, Başkent University Faculty of Medicine, Adana Dr. Turgut Noyan Application and Research Center,
  Adana, Turkey
4 Department of Dermatology, University Hospital “Santa Maria della Misericordia”, Udine, Italy



Citation: Tehçi T, Şenyurt EB, Durdu M, Errichetti E. Dermoscopic and Cytological Findings in Scleromyxedema. Dermatol Pract Concept.
2023;13(3):e2023147. DOI: https://doi.org/10.5826/dpc.1303a147
Accepted: December 4, 2022; Published: July 2023
Copyright: ©2023 Tehçi et al. This is an open-access article distributed under the terms of the Creative Commons Attribution-
NonCommercial License (BY-NC-4.0), https://creativecommons.org/licenses/by-nc/4.0/, which permits unrestricted noncommercial use,
distribution, and reproduction in any medium, provided the original authors and source are credited.
Funding: None.
Competing interests: None.
Authorship: All authors have contributed significantly to this publication.
Corresponding author: Murat Durdu, Department of Dermatology, Başkent University Faculty of Medicine, Adana Dr. Turgut Noyan
Application and Research Center, Adana, Turkey. Tel: +90 2422406710 Fax: +90 2422496040 Email: sivandr@hotmail.com




Case Presentation                                                        mucin deposition in the dermis (Figures 1, E and F). Lab-
                                                                         oratory tests showed monoclonal gammopathy, while no
A 63-year-old male presented with pruritic, firm, dome-                  systemic involvement was detected on further examination.
shaped, skin-colored/whitish papules mainly located over                 Intravenous immunoglobulin therapy (2 g/kg dose for 5 con-
the forehead, neck, elbows, hands and feet (Figure 1A) that              secutive days per month) was started, with significant im-
had progressively increased in number over the last year;                provement after three cycles of treatment.
skin induration of the trunk without papular lesions was
also evident on palpation. Dermoscopic examination of
                                                                         Teaching Point
the papules showed round/oval, homogenous, white-ivory
structureless areas similar to “rice grains” with no vessels             Scleromyxedema is a form skin mucinosis with possible
(Figure 1B) [1], while cytological assessment of slit-skin               extra-cutaneous involvement, including neurological, re-
smear taken from the lesions revealed round fibrotic colla-              nal, hematological, and rheumatological, that may carry a
gen structures and mucinous materials (Figures 1, C and D).              poor prognosis if not treated timely [2]. Diagnosis is gen-
Based on clinical, dermoscopic and cytological findings, a               erally clinical, yet in initial phases/incomplete instances
possible diagnosis of scleromyxedema was made, and a bi-                 it may be challenging to differentiate from similar condi-
opsy was taken for histological examination, that confirmed              tions (eg lichen planus, lichen amyloidosis, papular lichen
this hypothesis by revealing fibroblast proliferation, collagen          simplex chronicus, papular granuloma annulare, multiple
deposition, perivascular lymphoplasmocytic infiltration, and             follicular adnexal tumors) that, however, show a different




Image Letter | Dermatol Pract Concept. 2023;13(3):e2023147                                                                          1
    Figure 1. (A) Dome-shaped, firm, small papules on the nape are similar as seen on clinical examination. (B) Dermoscopy reveals
    round and oval-shaped white-ivory homogenous areas similar to rice grains (magnification x10) [1]. (C,D) Cytology shows round
    fibrotic collagen structures and mucinous materials (May-Grünwald Giemsa x1000). (E) Histopathological examination displays
    increased mucin deposition in the superficial dermis and stellated fibroblasts between collagen fibers (H&E x200). (F) Fragmented
    elastic fibers are also evident (Alcian blue stain x200).




2                                                                            Image Letter | Dermatol Pract Concept. 2023;13(3):e2023147
dermoscopic and cytological patterns [3]. Therefore, the use           scleromyxedema. JAAD Case Rep. 2019;5(5):451-453. Pub-
of such techniques may increase the index of suspicion for             lished 2019 May 8. doi:10.1016/j.jdcr.2019.03.005
                                                                    2. Ferreli C, Gasparini G, Parodi A, Cozzani E, Rongioletti F, At-
scleromyxedema with consequent prompt treatment.
                                                                       zori L. Cutaneous Manifestations of Scleroderma and Sclero-
                                                                       derma-Like Disorders: a Comprehensive Review. Clin Rev
References                                                             Allergy Immunol. 2017;53(3):306-336. DOI: 10.1007/s12016
                                                                       -017-8625-4. PMID: 28712039.
 1. Mendes Bastos P, Borges AS, Cardoso JC, Oliveira A. Dermos-
                                                                    3. Lallas A, Errichetti E, Ioannides D. Dermoscopy in General
    copy and reflectance confocal microscopy for the diagnosis of
                                                                       ­Dermatology. CRC Press, Boca Raton, FL, 2018.




Image Letter | Dermatol Pract Concept. 2023;13(3):e2023147                                                                          3
