Acta Dermato-Venereologica 99-10CompleteContent | Page 21

911 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