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To the Editors:
A 9-year-old boy presented with headaches and intermittent diplopia for approximately 3 weeks. His ocular history was unremarkable.
Ophthalmologic examination disclosed visual acuity of 10/10 in both eyes. He had 14 PD of right esotropia at near in primary position, which increased to 25 PD at distance. Limitation of right abduction with increased diplopia on right lateral gaze was noted (Fig. 1). Right lateral rectus palsy was confirmed on Lancaster red-green test and electro-oculography. There was no pain on eye movement.
Slit-lamp examination showed mild conjunctival erythema in the temporal quadrant of the right eye with no swelling of the conjunctiva or eyelids. Pupils were normal. Fundus examination yielded normal findings.
Orbital computed tomography, magnetic resonance imaging, and echography showed a marked enlargement of the right lateral rectus muscle belly, including the tendon, taking enhancement contrast in a homogeneous way, with no other soft tissue infiltration (Fig. 2).
On skin examination, patches of vitiligo were observed on the right part of the chin, the neck, and the inferior part of the cheek associated with a tuft of white hair on the right back of the neck and a few white eyelashes on the right superior lid. Scleroderma en coup de sabre was evident on the right forehead extending to the frontal scalp, causing alopecia. Two erythematous and alopecie patches were detected on the parietal scalp and upper auricular area. The superior part of the right external ear was red-purple with obvious thinning, evoking atrophie chondritis.
Results of the laboratory studies were normal, except for elevated serum immunoglobulin G, blood eosinophilia, and presence of rheumatoid factor. The antinuclear antibody test yielded weakly positive results with a homogeneous pattern, but antibodies to extractable nuclear antigen, particularly anti-doublestranded DNA and anti- Scl-70, were undetected.
Skin biopsy revealed a thickened and compact derma,...