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Unilateral corneal edema in young: A diagnostic dilemma

Scleroderma refers to a heterogeneous group of autoimmune fibrosing disorders. The disease can be either “localized” to the skin or “systemic” involving other organs in addition to the skin. Localized scleroderma can be further divided into linear and morphea (localized or generalized patches of atrophic and sclerotic skin lesions).[1] Linear scleroderma is characterized by localized fibrosis of skin, blood vessels, subcutaneous fat, muscle, and sometimes bone. It primarily affects the pediatric population in the first and second decade.[2] When linear scleroderma occurs on the fronto‐parietal scalp, it is referred to as “‘en coup de sabre,” given the resemblance of the skin lesion to the stroke of a sabre.[3] Specific neurological and ophthalmic associations of craniofacial scleroderma have been reported in literature that were all ruled out in our patient. The ophthalmic manifestations in scleroderma may include ptosis, lid induration or tightening, lash loss, motility disorder, keratoconjunctivitis sicca, conjunctival injection and vascular sludging, fornix shortening, iritis, heterochromia iridis, papillitis, retrobulbar pain, enophthalmos, displacement of outer canthus from resorption of orbital bone, and retinal hemorrhages.[2‐5]