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FP1123 : Ocular features in Hypohidrotic ectodermal dysplasia – A rare case

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FP1123 : Ocular features in Hypohidrotic ectodermal dysplasia – A rare case

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Dr.Himanshu gaikwad, G21426

Aim- to report the ocular association in a rare case of familial Hypohidrotic Ectodermal Dysplasia

Case report:- A 9 year old male presented in eye OPD with c/o OS swelling near medial canthus ×6 days and OD whiteness in cornea ×2 days. There is h/o OU on-off swelling in medial canthus ×5 years.

Va OD – CF 2 feet,Va OS – 6/24 ph-6/6 p ocular examination :- OD OS madarosis with OD corneal ulcer of size ~ 2×2 mm2 with surrounding infiltration of 0.5×0.5mm2 .OS showed swelling over medial canthus of 2×1 cm2

Schirmer OU decreased,TBUT OD- 4sec OS -5sec

Systemic examination-Hairs in scalp were sparse , hypopigmented ,and body hairs were absent. Skin was dry lustureless. Frontal bossing was present ,nasal bridge was depressed .OD ear CSOM was present . Few tooth were present.

Conclusion :- Hypohiderotic ectodermal dysplasia is a rare congenital anomaly. It manifests with dry eye ,madarosis. In our case presented with dry eye, madarosis and OD corneal ulcer with OS chronic dacryocystitis

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