A Rare Case of Pachydermoperisotosis (PDP) and Its Ocular Manifestations

Doi: 10.36351/pjo.v38i4.1399

Abstract

A case of 56 yearsold Pakistani male visited OPD with complaint of thickening of both eyelids, ptosis and left lower lid ectropion caused by rare condition named pachydermoperiostosis (PDP). PDP is a rare autosomal dominant condition but autosomal recessive families probably can also occur. It is manifested by clubbing of digits, hyperhidrosis of palm and feet, peri-ostosis, acro-osteolysis and pachydermia. Ocular features include blepharoptosis, floppy eyelids, eyelid and palpebral conjunctival hypertrophy, mechanical ectropion, meibomian gland dysfunction, tear film abnormalities, punctate epithelial erosions and ocular surface disease. Surgical management was given by full-thickness wedge resection leading to horizontal tightening and this was done along with shortening of levator and its advancement. Histopathology demonstrated chronic non-specific inflammation and foreign body giant cell reaction.