Ocular Histopathology of Acute and Chronic Pediatric Toxic Epidermal Necrolysis

Abstract

Purpose : Toxic Epidermal Necrolysis (TEN) can lead to devastating vision loss. Treatment of the ocular complications of TEN is predicated upon understanding the alterations of structure and function, and the pathophysiologic drivers thereof. Histopathology can yield powerful insights into all three of the above, but tissue specimens are collected infrequently and there is a time-limited window to observe the active, acute disease process. Here we report a series of 10 patients treated in whom sloughed tissue was submitted for histopathology.

Methods : We identified 10 patients recently treated for TEN for whom acute (<3 months from onset of skin slough) and/or chronic (3 months to 3 years after onset of skin slough) histopathology was available. Demographics and clinical data, including histology slides and pathology reports, were reviewed. Data are presented as average +/- standard error where appropriate.

Results : Twenty-seven specimens were identified from the 10 patients. These included 21 obtained within the acute timeframe and 6 obtained in the chronic timeframe. The acute specimens comprised sloughed tissue from 2 corneae, 8 conjunctivae, 2 mucocutaneous junctions, 6 eyelid outer lamellae excised as part of entropion repair, and 3 explanted amniotic membranes. The specimens obtained within the chronic timeframe included 4 eyelid excisions from entropion repair, 1 conjunctiva, and 1 eyelash line/mucocutaneous junction excision. The average age was 6 +/-1 years. Five of the patients were female. The most striking histopathological findings were loss of conjunctival epithelium in the acute specimens and minimal visualization of elastin fibers with Movat’s stain. This latter unexpected finding was observed in the conjunctival and eyelid excision tissue (Figure 1) and in specimens obtained during both the acute and chronic timeframes. An inflammatory infiltrate was observed in most acute specimens.

Conclusions : These findings provide insight into acute and chronic disease processes responsible for ocular pathology affecting pediatric TEN patients. Particularly the loss of outer eyelid lamellar elastin may explain the tendency of these patients to develop marked entropion. This, coupled with the well-recognized palpebral conjunctival cicatrization provides additive, rather than opposing force vectors that favor inward rotation of the lash line.

This is an abstract that was submitted for the 2018 ARVO Annual Meeting, held in Honolulu, Hawaii, April 29 - May 3, 2018.