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COMMENTARY

Ultrasound Morphea Activity Scoring


(US-MAS)
Modified US-MAS
Ximena Wortsman, MD
Department of Dermatology, School of Medicine, Pontificia Universidad Catolica de Chile, Santiago, Chile
Institute for Diagnostic Imaging and Research of the Skin and Soft Tissues, Santiago, Chile
Cristian Vera-Kellet, MD
Department of Dermatology, School of Medicine, Pontificia Universidad Catolica de Chile, Santiago, Chile

Morphea, an autoimmune connective tissue disease that affects the skin, can be
supported by color Doppler ultrasound in its diagnosis and assessment of activ-
ity. To date, there are no reliable laboratory parameters to track activity, and
ultrasound presents a higher axial spatial resolution than magnetic resonance
imaging and computed tomography, which is critical for studying the superficial
layers. The quality of the ultrasonographic assessment of activity in morphea
depends on the standardization and features of the acquisition of the anatomical
data. We propose a detailed ultrasound morphea activity scoring called modified
US-MAS (mUS-MAS) that could allow us to systematically register the cutane-
ous abnormalities in the corporal regions and their subregions. The selection of
the scanning sites will depend on the corporal regions of involvement and their
adjacent segments. Through systematic and sequential ultrasound data analysis,
we propose that this scoring system can better support description and activity
tracking accuracy.
Key Words—morphea; ultrasound; scleroderma; dermatologic ultrasound;
dermatology

M
orphea is an autoimmune connective tissue disease that
affects the skin and may be supported by ultrasound in
Manuscript accepted for publication
April 16, 2023.
its diagnosis and assessment of activity, which is relevant
The authors declare no conflicts of
because, so far, there are no reliable laboratory parameters that can
interest. help the tracking of activity, and the current clinical scorings of
Address correspondence to Ximena morphea may underestimate its subclinical involvement.1–4
Wortsman, MD, Institute for Diagnostic Moreover, ultrasound presents a higher axial spatial resolution
Imaging and Research of the Skin and Soft than other imaging modalities, such as magnetic resonance or
Tissues, Santiago, Chile; Department of
Dermatology, School of Medicine, Pontificia computed tomography, which is critical in the superficial tissues
Universidad Catolica de Chile, Santiago, and makes ultrasonography a first-line imaging technique in
Chile, Address: Lo Fontecilla 201 of 734, Las dermatology.5
Condes, Santiago, Chile.
In a publication of our group focused on evaluating the effi-
E-mail: xworts@yahoo.com
cacy of methotrexate, a first-line treatment in morphea, we applied
Abbreviations the Ultrasound Morphea Activity Scoring (US-MAS) to assess the
mUS-MAS, modified ultrasound morphea disease activity over time.6
activity scoring; US-MAS, arterial This scoring was based on the color Doppler examination of
blood gas
the corporal regions involved in morphea and their ultrasono-
doi:10.1002/jum.16247
graphic signs of activity already published in the literature.3,7–9

© 2023 American Institute of Ultrasound in Medicine. | J Ultrasound Med 2023; 9999:1–4 | 0278-4297 | www.aium.org
Wortsman and Vera-Kellet—Modified US-MAS Morphea Scoring

These activity signs include the increased subcutane- extremities; of them, only the face was divided into
ous echogenicity and/or loss of definition of the subregions, given its high cosmetic importance.
dermal-hypodermal border and the increase of the Nevertheless, the examination of each corporal
dermal and/or hypodermal vascularity.3,6,8 segment requires standardization of the exam proto-
However, this scoring was simplified and only col to not underestimate the actual activity of the dis-
considered macro corporal regions due to extensive ease in some areas.
data analysis in this study.6 Thus, in this initial US- Therefore, to improve the quality of the monitor-
MAS, the global activity assessment included the ing of the patients, we propose to perform a more
ultrasonographic sweep of the face, neck, trunk, and detailed ultrasound protocol that could allow the

Figure 1. A and B, Drawings of the regions and subregions of the corporal segments (surface). A, Anterior. B, Posterior. The decision on
the exact scanning sites will depend on the corporal regions where the clinical lesions and their adjacent corporal segments are located.

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Wortsman and Vera-Kellet—Modified US-MAS Morphea Scoring

follow-up of the activity of the subregions within the Figure 2. Signs of activity of morphea used in the mUS-MAS
(Modified Ultrasound Morphea Activity Score).
corporal regions. This new protocol is called modified
US-MAS (mUS-MAS).
In the ultrasound protocol, the study (ultrasono-
graphic sweep) involves the clinically affected corpo-
ral site (s) and their neighboring body regions to
evaluate the clinical and subclinical extent of the dis-
ease. Moreover, it has been reported that the cutane-
ous plaques can be asynchronous in their activity,
which supports the performance of systematized stud-
ies of the whole corporal regions and not just a single
or a few visible cutaneous plaques.10
Thus, for standardizing the sites of the color
Doppler ultrasound registrations, we propose record-
ing minimum sites at each corporal region and
subregion.
In the involvement of the limbs, the ultrasono-
graphic captures of imaging should be performed at
the upper, middle, and lower thirds of the dorsal and
ventral subregions of the arms, elbows, forearms,
wrists, and hands in the upper extremities, and the
thighs, knees, legs, ankles, and feet in the lower
extremities.
In the neck, the subregions can be scanned sepa-
rately according to the locations of the cutaneous
lesions in the anterior and posterior aspects. Each
aspect can be subdivided into the upper, middle, and
lower of the right, middle, middle, and left sides,
respectively.
In the trunk lesions, the subregions are divided
into the right, middle, and left anterior and dorsal
aspects of the thorax, which are subsequently
divided into upper, lower, and lower thirds. In In addition, each frontal and parietal region can be
women, the involvement of the breast regions can evenly divided into anterior and posterior, and the
be subdivided into right and left superior-external, occipital region can be subdivided into upper, middle,
superior-internal, inferior-external, and inferior- and lower following a similar scheme. The temporal
internal quadrants (subregions). subregions are subdivided into right and left.
The lumbar region can follow the same sequence In the affection of the face, the segments can be
type (right, middle, and left in the upper, middle, and separated into frontal, ears, nasal, cheeks, peribuccal,
lower thirds). The abdominal and pelvic walls can be lips, and chin, which can be subdivided according to
subdivided into right and left hypochondrium, flank, side; the frontal region is subdivided into right, mid-
iliac fossae, epigastrium, umbilical region, and hypo- dle, and lower thirds of the right, middle, and left
gastrium. The hips are divided into anterior and lateral regions. The nasal region can be divided into proxi-
aspects of the right and left sides. The gluteal regions mal, dorsum, and tip subregions. The lips are divided
are subdivided into quadrants (superior-external, into superior and inferior.
superior-internal, inferior-external, and inferior-internal). Drawings of the corporal regions and subregions
The scalp can be subdivided into the right, mid- of the anterior and posterior views of the body (sur-
dle, and left frontal, parietal, and occipital subregions. face) are shown in Figure 1. The ultrasonographic

J Ultrasound Med 2023; 9999:1–4 3


Wortsman and Vera-Kellet—Modified US-MAS Morphea Scoring

Table 1. Modified Ultrasound Morphea Activity Scoring Thus, a more detailed registration protocol can mean
(mUS-MAS)a a higher accuracy of the activity assessment over time.
Variable Score The IRB (Pontificia Universidad Catolica de
Chile) waived the need for informed consent to pub-
Increased subcutaneous (hypodermal) echogenicity and/or loss of
dermo-hypodermal bordersb
lish this commentary. The ultrasound images in
0 = negative Figure 2 belong to patients that provided informed
+2 = positive consent for publications.
Increase of dermal and/or subcutaneous (hypodermal) In conclusion, we believe that the modified US-
vascularization
0 = negative
MAS (mUS-MAS) can describe and follow up more
+2 = positive precisely on the corporal areas where the morphea
Type of flow inflammatory activity takes place.
0 = no increase in flow
+1 = venous or arterial <2 cm/s
+2 = arterial >2 cm/s References
Corporal regions extension
+1 = less than 2 corporal segments affected
+2 = 2 or more corporal segments affected 1. Fett N, Werth VP. Update on morphea: part II. Outcome mea-
Subregions within the corporal region’s extension sures and treatment. J Am Acad Dermatol 2011; 64:231–242.
+1 = less than 2 subregions affected within a corporal region https://doi.org/10.1016/j.jaad.2010.05.046.
+2 = 2 or more subregions affected within a corporal region 2. Fett NM. Morphea (localized scleroderma). JAMA Dermatol 2013;
Variables Added in Control CDU (Compared With Previous) 149:1124. https://doi.org/10.1001/jamadermatol.2013.5079.
3. Wortsman X, Wortsman J, Sazunic I, Carreno L. Activity assess-
Increase in size of affected subregions and/or corporal segments
+1 = increase in the size of 1 affected subregion ment in morphea using color Doppler ultrasound. J Am Acad
+2 = increase in the size of 2 or more affected subregions Dermatol 2011; 65:942–948. https://doi.org/10.1016/j.jaad.2010.
+2 = extension in the size of the same affected subregion or to 08.027.
another corporal segment 4. Marti-Marti I, Morgado-Carrasco D, Podlipnik S, et al. Usefulness
Appearance of new affected subregions in the same or different
of high-frequency ultrasonography in the evaluation and monitor-
corporal segments
0 = negative ing of sclerosing dermatoses: a cohort study. Clin Exp Dermatol
+2 = positive 2022; 47:351–358. https://doi.org/10.1111/ced.14903.
Decrease in maximum size or number of affected subregions 5. Wortsman X. Top advances in dermatologic ultrasound. J Ultrasound
0 = negative Med 2023; 42:521–545. https://doi.org/10.1002/jum.16000.
1 = positive, partially
6. Vera-Kellet C, Meza-Romero R, Moll-Manzur C, Ramírez-
2 = positive, completely
Maximum Scoring: 16 points Cornejo C, Wortsman X. Low effectiveness of methotrexate in the
management of localised scleroderma (morphea) based on an
a
The analysis of each body segment is subdivided in body regions
ultrasound activity score. Eur J Dermatol 2021; 31:813–821.
according to text. https://doi.org/10.1684/ejd.2021.4189.
b
In control CDU, this item can be considered as +1 point when 7. Laverde-Saad A, Lopez-Negrete E, Roustan G, Alfagemeb F.
echogenicity or vascularization remain altered but have partially Dermatologic ultrasound in the management of childhood linear
improved. morphea. Dermatol Online J 2021; 27(7). https://doi.org/10.
5070/d327754364.
8. Wortsman X. Why, how, and when to use color Doppler ultra-
signs of activity considered in the modified US-MAS sound for improving precision in the diagnosis, assessment of
are demonstrated in Figure 2. severity and activity in morphea. J Scleroderma Relat Disord 2019;
This modified US-MAS scoring uses similar 4:28–34. https://doi.org/10.1177/2397198318799244.
parameters of the already published US-MAS but 9. Zhang S, Zhu QL, Xiao MS, Liu J. The value of dermoscopy and
adds the subregional involvement in all the corporal high-frequency ultrasound in staging morphea. J Dermatol 2022;
segments (Table 1). This could locate and track 50(4):511–517. https://doi.org/10.1111/1346-8138.16648.
much better the anatomical location of subtle subclin- 10. Wortsman X. Textbook of Dermatologic Ultrasound. 1st ed. New
ical changes and support research and clinical trials. York, NY: Springer; 2022.

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