PMC8102938_01
(a) Right eye shows an oval area of deep scleral thinning without uveal prolapse, adjacent to conjunctival hyperemic zone in the interpalpebral area. (b) Left eye shows an area of conjunctivalized thinned scleral in the interpalpebral area
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F1
Slit-lamp biomicroscopy revealed an oval area of deep scleral thinning without uveal prolapse, adjacent to a conjunctival hyperemic zone in the interpalpebral area, 2 mm temporal to the limbus [Figure 1a] in the right eye. The left eye presented with a conjunctivalized scleral thinning in the interpalpebral area, 2 mm temporal to the limbus [Figure 1b].
Hyperpigmentation and hypertrichosis on the face
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F2
Physical examination yielded facial hyperpigmentation and hypertrichosis [Figure 2] and multiple hypopigmented scars in hands and nails [Figure 3].
Multiples hypopigmented scars on the skin dorsum hand
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F3
Physical examination yielded facial hyperpigmentation and hypertrichosis [Figure 2] and multiple hypopigmented scars in hands and nails [Figure 3].
(a and b) Improvement of scleral lesions in both eyes at the last follow-up visit
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F4
Slit-lamp biomicroscopy revealed a decrease in the size and depth of the scleral lesions, as well as a reduction of conjunctival hyperemia adjacent to the lesion in the right eye [Figure 4].