Dr. Karan Bhatia, B15311, Dr. PraveenDhanapal, Dr. Seema Ramakrishnan, Dr. Saloni Umesh Shah
Background
IFAP is a rare X-linked disorder characterized by triad of follicular ichthyosis, atrichia of scalp, eyebrows, eyelashes and photophobia with 40 reported cases so far.
Case Report
A 21 year old man born of consanguineous parents presented with recurrent redness, irritation in both eyes (BE), photophobia, decreased sweating since many years, alopecia totalis and ichthyosis of scalp, flexor and extensor surfaces of forearm extending to arms, sparing the plantar areas. He had madarosis, absent eye brows, mandibular prognathism, gingival recession with hypodontia, but without any hearing problems, with similar problems in few male members of family. Ocular examination showed superficial punctate keratitis with a subepithelial paracentral corneal opacity, without any vascularization in BE. He was managed with topical steroids, lubricating eye drops and retinol palmitate injections.
Conclusion
Early recognition along with genetric counselling plays a key role in its management.