Histopathologic examination of the skin and eyelids reveals parakeratosis of the stratum corneum with occasional neutrophils and intracellular edema. The granular cell layer is diminished, and the upper epidermis demonstrates pallor and edema. Focal dyskeratosis is seen. The epidermis may be psoriasiform or atrophic. One case report details the ocular histopathology of a child who died before efficacious treatment was available.
Systemic treatment requires lifelong zinc supplementation.
Ocular therapy requires vigorous ocular surface supportive measures and intervention when secondary bacterial or candidal infection, trichiasis, or other complications occur. Advanced cases may require oculoplastic surgical intervention including lid reconstruction, tarsorrhaphy, or epilation therapy. Ocular surface reconstruction with conjunctival transplantation or human amniotic membrane grafts also is a possibility in severe situations. Corneal transplantation may be required in cases of severe keratomalacia or infectious keratitis. Consultation with pediatrics, genetics, dermatology, or plastic surgery may be useful.