Ocular involvement in lamellar Ichthyosis

Amit Gupta¹ , Aniruddha Agarwal² , Jagat Ram¹

¹ Department of Ophthalmology, Advanced Eye Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India
² Department of Ophthalmology, Advanced Eye Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India; Ocular Imaging Research and Reading Center, Stanley M. Truhlsen Eye Institute, University of Nebraska Medical Center, Omaha, USA,

Correspondence Address:
Jagat Ram
Advanced Eye Centre, Department of Ophthalmology, Post Graduate Institute of Medical Education and Research, Chandigarh - 160 012
India

A 25-year-old male with lamellar ichthyosis presented with decreased vision in the left eye for 1 month. There was extensive scaling and hyperkeratosis involving the whole body, coarse and sparse scalp hair and right genu valgus [Figure - 1]a. Tightening of facial skin resulting in cicatricial ectropion, thick edematous eyelid, lateral madarosis, erythema and distortion of conjunctival architecture, clouding and edema of the cornea due to incomplete lid closure were evident on examination of the eye [Figure - 1]b. Despite aggressive therapy with emollients, ocular lubricants and systemic isotretinoin, the patient developed total corneal opacity with vascularization. Early diagnosis and intervention for ocular complications may prevent visual loss in these patients.

Figure 1: (a) Edematous eyelid, lateral madarosis, erythema and corneal oedema due to incomplete lid closure. (b) Extensive scaling and hyperkeratosis involving the whole body, coarse, and sparse scalp hair and right genu vulgus