10.5826/dpc.1203a142
Introduction: Dermoscopy is a noninvasive and easy to apply technique that allows in vivo magnification of the skin and thus observation of morphologic structures invisible to the naked eye. Recently, it gained popularity for evaluation of inflammatory skin conditions. In the field of connective tissue diseases, dermoscopy has been used mainly as a simple and accessible substitute of nailfold capillaroscopy.
Objectives: The aim of the present study is to expand the application of dermoscopy in patients with dermatomyositis (DM) beyond the usual nailfold examination. A clinico-dermoscopic correlation is also suggested.
Methods: A total of twenty-nine patients with DM were enrolled in this descriptive prospective study, conducted over a three-year period. Dermoscopy was performed by a DermLite DL1 dermatoscope on polarization mode, attached to One Plus 3T camera. The following skin lesions were examined - periungual affection, scalp DM, Gottron’s papules, palmar papules, poikiloderma and auricular changes.
Results: Dermoscopy detected predominantly advanced nail fold capillary changes - giant capillaries (79%), microhemorrhages (46%) and avascular areas (25%). The most prevalent trichoscopic features were enlarged tortuous capillaries (64%), interfollicular scales (50%) and peripilar casts and tufting (36%). Among the other skin lesions assessed in this study - Gottron's papules were present in 20 patients, poikiloderma in 11, palmar papules in 4 and auricular lesions in 4 patients.
text/xml
2160-9381
2067
en
Lyubomir  Dourmishev
https://creativecommons.org/licenses/by-nc/4.0
Dermatology Practical & Conceptual
dermoscopy, dermatomyositis, scalp dermatomyositis, palmar papules, auricular lesions
Cutaneous Manifestations and Their Corresponding Dermoscopic Features in Patients with Dermatomyositis
Text.Serial.Journal
https://dpcj.org/index.php/dpc
Original Article
2022-07-28
2021-09-23
2022-07-28
2022-09-20
e2022142
Dermatol Pract Concept
Introduction: Dermoscopy is a noninvasive and easy to apply technique that allows in vivo magnification of the skin and thus observation of morphologic structures invisible to the naked eye. Recently, it gained popularity for evaluation of inflammatory skin conditions. In the field of connective tissue diseases, dermoscopy has been used mainly as a simple and accessible substitute of nailfold capillaroscopy.
Objectives: The aim of the present study is to expand the application of dermoscopy in patients with dermatomyositis (DM) beyond the usual nailfold examination. A clinico-dermoscopic correlation is also suggested.
Methods: A total of twenty-nine patients with DM were enrolled in this descriptive prospective study, conducted over a three-year period. Dermoscopy was performed by a DermLite DL1 dermatoscope on polarization mode, attached to One Plus 3T camera. The following skin lesions were examined - periungual affection, scalp DM, Gottron’s papules, palmar papules, poikiloderma and auricular changes.
Results: Dermoscopy detected predominantly advanced nail fold capillary changes - giant capillaries (79%), microhemorrhages (46%) and avascular areas (25%). The most prevalent trichoscopic features were enlarged tortuous capillaries (64%), interfollicular scales (50%) and peripilar casts and tufting (36%). Among the other skin lesions assessed in this study - Gottron's papules were present in 20 patients, poikiloderma in 11, palmar papules in 4 and auricular lesions in 4 patients.
Lyubomir  Dourmishev
2022/07/28
10.5826/dpc.1203a142
e2022142
Department of Dermatology and Venereology, Medical University, Sofia, Bulgaria
2160-9381
dermoscopy, dermatomyositis, scalp dermatomyositis, palmar papules, auricular lesions
en
e2022142
Cutaneous Manifestations and Their Corresponding Dermoscopic Features in Patients with Dermatomyositis
https://dpcj.org/index.php/dpc/article/download/2067/1698
                    Dermatology Practical & Conceptual


Cutaneous Manifestations and Their Corresponding
      Dermoscopic Features in Patients with
               Dermatomyositis
                        Joana Pozharashka1, Ljubka Miteva1, Lyubomir Dourmishev1


1 Department of Dermatology and Venereology, Medical University, Sofia, Bulgaria



Key words: dermoscopy, dermatomyositis, scalp dermatomyositis, palmar papules, auricular lesions
Citation: Pozharashka J, Miteva L, Dourmishev L. Cutaneous manifestations and their corresponding dermoscopic features in patients with
dermatomyositis Dermatol Pract Concept. 2022;12(3):e2022142. DOI: https://doi.org/10.5826/dpc.1203a142
Accepted: December 23, 2021; Published: July 2022
Copyright: ©2022 Pozharashka 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: Lyubomir Dourmishev MD, PhD, 1 St. Georgi Sofiiski Blvd., Sofia 1431, Bulgaria, Phone: +359 2 9230438
E-mail: l_dourmishev@yahoo.com




  ABSTRACT             Introduction: Dermoscopy is a noninvasive and easy to apply technique that allows in vivo magnifica-
                       tion of the skin and thus observation of morphologic structures invisible to the naked eye. Recently, it
                       gained popularity for evaluation of inflammatory skin conditions. In the field of connective tissue dis-
                       eases, dermoscopy has been used mainly as a simple and accessible substitute of nailfold capillaroscopy.
                       Objectives: The aim of the present study is to expand the application of dermoscopy in patients with
                       dermatomyositis (DM) beyond the usual nailfold examination. A clinico-dermoscopic correlation be-
                       tween clinical signs of skin affection and dermoscopic features is also suggested.
                       Methods: A total of 29 patients with DM were enrolled in this descriptive prospective study, conduct-
                       ed over a 3-year period. Dermoscopy was performed by a DermLite DL1 dermatoscope on polariza-
                       tion mode, attached to One Plus 3T camera. The following skin lesions were examined: periungual
                       affection, scalp DM, Gottron papules, palmar papules, poikiloderma and auricular changes.
                       Results: Dermoscopy detected predominantly advanced nail fold capillary changes - giant capillaries
                       (79%), microhemorrhages (46%) and avascular areas (25%). The most prevalent trichoscopic features
                       were enlarged tortuous capillaries (64%), interfollicular scales (50%) and peripilar casts and tufting
                       (36%). Among the other skin lesions assessed in this study - Gottron papules were present in 20 pa-
                       tients, poikiloderma in 11, palmar papules in 4 and auricular lesions in 4 patients.
                       Conclusions: The use of dermoscopy for clinical evaluation of skin lesions in DM enhances diagnostic
                       accuracy and elucidates poorly known characteristics of the disease.




Original Article | Dermatol Pract Concept. 2022;12(3):e2022142                                                                        1
Introduction                                                       DM/overlap syndrome with another connective tissue dis-
                                                                   ease (3 patients), such as systemic lupus erythematosus and
Dermoscopy (epiluminescence microscopy) is a noninvasive           systemic sclerosis were enrolled in this prospective descrip-
and easy to apply technique that allows in vivo magnification      tive study. The study was conducted in the Department of
of the skin and observation of morphologic features invisible      Dermatology and Venereology, Alexandrovska University
to the naked eye. In the last decades dermoscopy has become        Hospital, Sofia, Bulgaria between September 2018 and
a key tool for the evaluation of pigmented and nonpigmented        August 2021. Only patients with diagnosis of definite DM
skin tumors. Recently, an increasing number of publications        according to Bohan and Peter criteria for classic DM were
have appeared that demonstrate the role of the handheld der-       included [5]. Patients with amyopathic DM were selected
matoscope in an entirely new field - the field of inflammatory     based on clinical skin findings, suggested by Sontheimer
skin lesions. A huge break-through in this direction was the       [6,7]. Additional examinations comprised serum enzyme lev-
expert consensus published in 2020 on behalf of the Interna-       els, myositis-specific autoantibodies, electromyography, and
tional Dermoscopy Society providing a set of standardized          skin biopsy. The mean age was 51 years (range: 19-77 years).
basic dermoscopic parameters to follow when evaluating in-         Twenty patients were females (69%) and 9 patients were
flammatory, infiltrative and infectious dermatoses [1].            males (31%). A cancer-associated, or paraneoplastic DM
    Nailfold capillaroscopy (NFC) is currently considered the      was diagnosed in 10 patients (breast carcinoma in 2 patients,
gold standard for early assessment of microvascular changes        cervical carcinoma in 2, thyroid carcinoma in one, endome-
in the nailfold area of patients with rheumatic diseases. As a     trial carcinoma in 2, ovarian carcinoma in one, cecal carci-
simpler and more accessible method than NFC, dermoscopy            noma in one, and bladder carcinoma in one). The disease
has also been tested for gross analysis of capillary nailfold      duration ranged between 1 month and 23 years, and 69%
abnormalities in several studies comprising patients with col-     of the patients had a maximal disease duration of 3 years.
lagen-vascular disorders [2,3].                                       All the 29 patients were included in the study after sign-
    Dermatomyositis (DM) is a rare autoimmune connective           ing a written informed consent.
tissue disease that affects the skin, the skeletal muscles and        The dermoscopic examination was performed by a Der-
the internal organs. Although its pathogenesis is not fully un-    mLite DL1 dermatoscope on polarization mode, attached to
derstood, the immune response is thought to originate from         One Plus 3T camera.
the capillary endothelium of the endomysium. An activation
of the complement pathway and deposition of C5b-9 mem-             Results
brane attack complexes results in depletion of capillaries,
ischemia, muscle and skin injury [4]. Therefore, vascular skin     Affection of periungual skin was assessed by the following
changes are fundamental for DM and a variety of vascular           clinical parameters-ragged cuticles, erythema, nail fold telan-
patterns might be observed via dermoscopy.                         giectasias visible by naked eye, erosions/ulcerations, skin at-
                                                                   rophy. Ragged cuticles (N = 14,58%) and nail fold erythema
                                                                   (N = 14,58%) were relatively common in our patients. Some
Objectives                                                         patients had only one of these parameters, and some of them
The aim of the present study is to expand the application of       had both. Macroscopic nail fold telangiectasias (N = 1),
dermoscopy in patients with DM beyond the simple nailfold          erosions/ulcerations (N = 2) and skin atrophy of the periun-
examination. In order to do that, we have performed dermos-        gual area (N = 1) were a rare finding (supplementary Table
copy of other skin lesions, such as scalp DM, Gottron pap-         S1; Figure 1).
ules, palmar papules, poikiloderma and auricular lesions in the       Specific dermoscopic features were divided into 5 groups -
quest for subtle diagnostic clues, invisible to the unaided eye.   subtle changes or disorganization of the normal capillary
In addition, we suggest a clinico-dermoscopic correlation be-      architecture, enlarged/giant capillaries, microhemorrhages
tween clinical signs of skin affection and dermoscopic features.   (defined by > 2 microhemorrhages per digit or confluent
    Due to the low incidence of DM in the population, der-         hemorrhagic zones), loss of capillaries/large avascular ar-
moscopic data in different studies is combined with data           eas, and ramified/bushy capillaries. Most of the patients had
from other connective tissue diseases which compromises            more pronounced nail fold capillary changes therefore only
the unique characteristics of the disease. The present study       three patients presented with disorganization of the normal
focuses only on patients with DM.                                  capillary architecture (13%). Enlarged/giant capillaries were
                                                                   the most common dermoscopic feature and occurred in nine-
                                                                   teen patients (79%). Other signs like microhemorrhages,
Methods
                                                                   avascular areas and bushy capillaries were found in 46%
Twenty-nine patients with classic DM (CDM, 22 pa-                  (N = 11), 25% (N = 6) and 13% (N = 3) of the patients
tients), clinically amyopathic DM (CADM, 4 patients) and           respectively (supplementary Table S1; Figure 11).


2                                                                      Original Article | Dermatol Pract Concept. 2022;12(3):e2022142
Figure 1. (A and B) Disorganization of the normal capillary architecture. (C-G) Enlarged/giant capillaries (gray arrows), microhemorrhages
(red arrows), loss of capillaries/large avascular areas (blue arrows). (H and I) Ramified/bushy capillaries (purple arrows). (J) Nailfold erosion
covered with crust. (K and L) Ragged cuticles.



    Clinical signs of scalp affection comprised erythema                      Poikiloderma, which by definition encompasses telangi-
(N = 12, 55%), scaling (N = 11, 50%), non-scarring alope-                 ectasias, dyspigmentation and atrophy, was found in eleven
cia (N = 14, 64%), as well as calcinosis cutis and erosions/              patients (38%) and palmar papules in four patients (14%).
ulcerations (none of the patients). Pruritus was reported by                  Dermoscopic data about Gottron papules, poikiloderma
16 patients (73%).                                                        and palmar papules is presented in supplementary Table S1
    Dermoscopy of the scalp, also called trichoscopy, revealed            and Figure 3.
enlarged tortuous capillaries in fourteen patients (64%),                     Several unusual findings which deserve attention were
peripilar casts and tufting in eight patients (36%), interfollic-         also noticed. Four of the patients had auricular lesions
ular scales in eleven patients (50%), bushy capillaries in three          (prominent telangiectasias, venous lake-like structures, ero-
patients (14%), interfollicular pigmentation in five patients             sions). One patient presented with significant perifollicular
(23%), perifollicular pigmentation in two patients (9%), vas-             erythema on the body and extremities (supplementary Table
cular lake-like structures in seven patients (32%), punctuate             S1 and Figure 3).
hemorrhages in one patient (5%). Hair shaft abnormalities
were also detected - broken hairs (n=2; 9%), pigtail hairs (n=1;
                                                                          Conclusions
5%), in the absence of other conditions, that may present with
such hair defects (e.g., fungal infection, alopecia areata etc.).         Periungual changes are a well-known marker for cutaneous
Clinical and dermoscopic parameters concerning scalp affec-               affection in DM. As a standard practice, periungual lesions
tion are shown on supplementary Table S1 and Figure 2.                    are examined via NFC. NFC pattern in DM is generally sim-
    Dermoscopy of Gottron papules visualized erythema                     ilar to this found in systemic sclerosis patients and therefore
(N = 20, 100%), scaling (N = 20, 100%), skin atrophy                      is referred to as a “scleroderma-like pattern”. NFC rep-
(N = 1, 5%), irregularly arranged vessels (N = 6, 30%) and                resents a valuable tool for the clinician as studies confirm
erosions/ulcerations (N = 1, 5%).                                         that changes in nail-fold capillaries reflect disease activity in


Original Article | Dermatol Pract Concept. 2022;12(3):e2022142                                                                                 3
Figure 2. (A and B) enlarged tortuous capillaries. (C and D) Tufting (purple arrows), peripilar casts (blue arrows), perifollicular pigmentation
(gray arrow). (E) Interfollicular scales. (F) Interfollicular pigmentation. (G) Vascular lake-like structure. (H) Bushy capillaries. (I) Perifollicular
erythema. (J) Punctuate hemorrhages. (K) Broken hairs; (L) Pigtail hairs.


DM [8,9]. Data on periungual dermoscopy in DM patients                       present [13-15]. We found only one study that examines
is scarce and it is unclear whether dermoscopy might be used                 scalp dermoscopy in DM patients [16]. In this study the au-
as a prognostic method for disease activity, in the same way                 thors performed trichoscopy on 31 patients with DM and
as NFC, in those patients.                                                   concluded that in their patient group the most consistent
    Our dermoscopic findings correspond roughly to those re-                 finding was the presence of enlarged capillaries (71.4%), fol-
ported in the literature [10-12]. Most of the patients presented             lowed by peripilar casts (57.1 %) and tufting, and interfollic-
with scleroderma-like pattern suggestive for active disease.                 ular scaling (50%). Our results demonstrated predominance
The most frequent nailfold dermoscopy features were respec-                  of the same dermoscopic features - enlarged tortuous capil-
tively enlarged or giant capillaries and microhemorrhages.                   laries in fourteen patients (64%), peripilar casts and tufting
The enlarged/giant capillaries are regarded as an abnormal                   in eight patients (36%), interfollicular scales in eleven pa-
angiogenic response, secondary to peripheral ischemia. Hem-                  tients (50%). In addition, we detected some hair shaft abnor-
orrhages most probably result from capillary injury caused                   malities (broken hairs, pigtail hairs) in the absence of other
by ischemia-reperfusion. Signs of early (disorganization of the              conditions that may present with such hair defects. We did
normal capillary architecture) or late scleroderma-like pattern              not find any previous publications about hair shaft abnor-
(large avascular areas/ loss of capillaries and ramified/bushy               malities in patients with DM.
capillaries) were less frequent in our patient group.                             Enlarged capillaries are a trichoscopic finding character-
    Scalp involvement is a generally overlooked clinical char-               istic for connective tissue diseases. They can be easily dif-
acteristic of DM hence its actual frequency remains unrecog-                 ferentiated from other vascular patterns, such as prominent
nized. In the majority of manuals and review articles scalp                  arborizing vessels in seborrheic dermatitis or twisted capil-
DM is described as an erythematous, scaly and sometimes                      lary loops in psoriasis [17].
pruriginous condition of the scalp, resembling seborrheic                         Hair tufting usually is regarded as a sign of scarring
dermatitis or psoriasis. Often nonscarring alopecia is also                  alopecia. The presence of more than six hairs suggests a



4                                                                                  Original Article | Dermatol Pract Concept. 2022;12(3):e2022142
  Figure 3. (A) Ulcerative Gottron papules. (B and C) Dermoscopy of Gottron papules. Note the circular appearance of the enlarged
  vessels in (C).Clinical (D) and dermoscopic pictures of palmar papules (E-G). Note how palmar papules are located on the two op-
  posite sides of the joint.,(H) Poikiloderma on dermoscopy. (I) Perifollicular erythema on dermoscopy. (J) Auricular erosions. (K and L)
  Telangiectasias and vascular lake-like structures of the auricle on dermoscopy.



diagnosis of folliculitis decalvans. Hair tufts in lichen plano-       addressed. Further prospective studies are needed to elab-
pilaris contain fewer than six hairs (small tufts of four to five      orate a guideline for evaluation of trichoscopic features in
hairs) . For the purpose of this study, we consider tufting
      17
                                                                       inflammatory diseases, especially considering rare diseases
as more than 3 hair shafts emerging together from the same             such as DM [1].
follicular opening, similarly to inflammatory scalp diseases.              Dermoscopic picture of Gottron papules and Got-
    Tufts tend to be surrounded by peripilar casts or tube-            tron sign is nonspecific. Irregular capillaries and scales on
like layers of scales enclosing the hair shafts. The latter are        an erythematous background could be easily visualized.
also considered indicative of scarring alopecia [17].                  However, single peculiar cases are described. Hasegawa re-
    Broken hairs are relatively non-specific and are seen in           ported the presence of punctuate hemorrhages, detected by
a variety of scalp diseases, such as alopecia areata, tricho-          dermoscopy, on the elbow of a patient with Gottron sign
tillomania, tinea capitis, and trichorrhexis nodosa. In alo-           [11]. The same patient was positive for anti-melanoma dif-
pecia areata, broken hairs may develop by two mechanisms.              ferentiation-associated protein 5 (MDA-5) autoantibody and
One is inflammation-driven transverse fracture of terminal             later-on developed a rapidly progressive interstitial lung dis-
hair shafts and the other, rapid regrowth of incompletely de-          ease. Hasegawa suggested that punctate hemorrhages might
stroyed hair shafts [14].                                              be related to vascular injury in the context of the aggressive
    Pigtail or circular hairs appear as a result from fast hair        interstitial pneumonia. The correlation between anti-MDA
regrowth before full recovery of the hair follicle. They are           5 antibody specificity in DM patients and the presence of
considered typical for alopecia areata although sometimes              skin ulcers is well known. Despite that, ulcerative Gottron
found in cicatricial alopecia [14].                                    papules/Gottron sign are a rather rare phenomenon [18]. In
    In the expert consensus on dermoscopic parameters in               our patient group we had a case with anti-MDA 5 autoanti-
general dermatology the topic about scalp dermoscopy is not            bodies who exhibited ulcerated Gottron papules, noticeable



Original Article | Dermatol Pract Concept. 2022;12(3):e2022142                                                                              5
by naked eye, along with ulcerations all over the body and                 The use of dermoscopy for clinical evaluation of skin
extremities (Figure 3).                                              lesions in DM enhances diagnostic accuracy and elucidates
    Palmar papules or inverse Gottron papules are tender             poorly known characteristics of the disease.
and often painful lesions on palmar surfaces with a predi-                 The present study had some obvious limitations due to
lection towards the metacarpal and interphalangeal joints.           its small sample size. Larger studies could help to distinguish
They are described also as a part of the MDA-5 antibody              clinical and dermatoscopic hallmarks in various subtypes of
phenotype. Sometimes they are two separate papules on the            DM. The role of dermoscopy as a prognostic tool for dis-
two opposite sides of the joint [19]. Dermoscopy of palmar           ease activity and outcome in patients with DM remains to
papules is virtually unexplored. In our patient group we had         be investigated.
four patients with palmar papules examined by dermoscopy.
We noticed the presence of dotted vessels on an erythema-
tous base, sometimes with an orange hue. Scales were also            References
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Original Article | Dermatol Pract Concept. 2022;12(3):e2022142                                                                             7
