Acta Dermato-Venereologica 99-10CompleteContent | Page 21
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SHORT COMMUNICATION
Acquired Strabismus in Linear Scleroderma of the Face
Giorgia MARTINI 1 , Maria Elisabetta ZANNIN 2 , Fiorella RODEGHIERO 3 , Sandro DAL POS 4 and Francesco ZULIAN 1
Paediatric Rheumatology Unit, 2 Paediatric Ophthalmology Unit, Department of Woman and Child Health, 3 Ophthalmology Department, and
Neuroradiology Department, University of Padova, IT-35128 Padova, Italy. E-mail: [email protected]
1
4
Accepted Jun 13, 2019; E-published Jun 14, 2019
Juvenile localized scleroderma (JLS) comprises a group
of autoimmune fibrosing conditions involving the skin
and subcutaneous tissues following an initial inflam-
matory reaction. Linear scleroderma (LiS) is the most
common subtype, characterized by one or more linear
streaks of fibrosis, which can involve the dermis, subcuta-
neous tissue, muscle and, sometimes, bone (1). Although
JLS is not a fatal disease, affected children may develop
severe functional sequelae, such as joint contractures,
limb growth discrepancy and psychological problems
due to cosmetic disfiguration. Approximately one-quarter
of patients present extracutaneous complications (2–4).
Therefore, prompt diagnosis and treatment are necessary
in order to reduce disease activity and halt tissue damage.
We report here a girl who presented acquired strabismus
as an unusual complication of LiS affecting the face.
CASE REPORT
A 3-year-old girl developed a hyperchromic lesion with an ivory-
like centre on the lateral aspect of her right eyebrow. The lesion
subsequently extended to the temporal area, and 2 years later she
presented intermittent exotropia. Cerebral computed tomography
(CT) scan was normal and no treatment was advised. After 4
years she was referred to our unit and finally, at 7 years of age,
a diagnosis of LiS of the face was made. On examination she
presented right exotropia and hypotropia due to impairment of
superior, medial and inferior rectus and superior oblique muscles.
A linear violaceous lesion with moderate subcutaneous tissue
loss and adnexa atrophy was present on the
temporal area with small ivory-like centre.
Cerebral magnetic resonance imaging (MRI)
was performed and confirmed focal atrophy of
subcutaneous fat tissue on the temporal area,
compatible with scleroderma lesion, thinness
and atrophy of superior, medial and inferior
rectus and superior oblique muscles, but no
cerebral abnormalities (Fig. 1). Treatment
with methotrexate was commenced, and was
continued for 2 years, in combination with
prednisone for the first 3 months. A definite
improvement in the skin lesion, with disap-
pearance of inflammation and softening with
hyperpigmentation, was observed with this
treatment, while impairment of the extraocular
muscles remained the same (Fig. 2).
Fig. 1. Coronal T2-weighted magnetic resonance imaging (MRI)
image showing focal subcutaneous tissue loss in the right fronto-parietal
region (arrowhead) and atrophy of the extraocular muscles of right orbit:
superior, medial and inferior rectus (black arrows) and superior oblique
(white arrow).
JLS is not known, but there is evidence that the majority
of cases occur during childhood (mean age at onset 7.9
years) (5, 6).
LiS is the most common form of JLS and, when oc-
curring on the head, is referred as “en coup the sabre”
(ECDS) due to the resemblance of the skin lesions to
DISCUSSION
LiS is a rare disease, with an estimated
annual incidence of 0.4–2.7 per 100,000
individuals. The overall incidence of
Fig. 2. (A) Skin lesion on the temporal area with hyperpigmentation and subcutaneous tissue
atrophy (black arrow). (B) Right eye exotropia with elevation impairment. (C) Hyperfunction of
superior oblique muscle causing elevation and adduction of the left eye. (D) Impairment of medial
rectus muscle of the right eye. Written permission from the mother is given to publish these photos.
This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta
Journal Compilation © 2019 Acta Dermato-Venereologica.
doi: 10.2340/00015555-3238
Acta Derm Venereol 2019; 99: 911–912