Periorbital scleroderma associated with heterochromia iridis

Area of Science:

• Ophthalmology
• Dermatology
• Connective Tissue Diseases

Background:

• Linear scleroderma is a localized scleroderma variant characterized by linear bands of skin hardening.
• Ocular manifestations of scleroderma are uncommon but can be severe.
• Pigmentary abnormalities in the anterior segment of the eye are rarely reported in association with scleroderma.

Observation:

• Two patients with biopsy-proven linear scleroderma presented with distinct ocular pigmentary abnormalities.
• Patient 1 exhibited heterochromia iridis, upper eyelid atrophy, a coup de sabre lesion, and asymmetric pigmentary glaucoma.
• Patient 2 had bilateral scalp involvement, extraocular muscle paresis, blepharoptosis, and heterochromia iridis.

Findings:

• The study identifies a potential association between linear scleroderma and anterior segment pigmentary abnormalities.
• Specific ocular findings included heterochromia iridis, eyelid changes, and pigmentary glaucoma.
• Neuromuscular involvement, such as extraocular muscle paresis, was also noted in one patient.

Implications:

• This association suggests a need for comprehensive ophthalmologic evaluation in patients diagnosed with linear scleroderma.
• Early detection of ocular complications may allow for timely intervention and management.
• Further research is warranted to elucidate the pathogenesis of these ocular findings in linear scleroderma.