Extracutaneous manifestations and long-term sequelae of Stevens-Johnson syndrome/toxic epidermal necrolysis

This month sees the publication of an important piece of research in the field of medical dermatology, specifically in the domain of Stevens–Johnson syndrome and toxic epidermal necrolysis (SJS‐TEN).1 With the imminent publication of the British Association of Dermatologists’ guidelines on the management of this disease spectrum, this paper provides a timely opportunity to reflect on an important extracutaneous aspect of SJS‐TEN, currently under‐recognized by clinicians both within and outside of dermatology. The provenance of the data is the world‐renowned tertiary referral centre for the management of severe cutaneous adverse reactions, Hôpital Henri Mondor, which has been the professional home of many of the names now synonymous with clinical research in SJS‐TEN: Roujeau, Revuz and Bastuji‐Garin to name a few. Previously published work has demonstrated that approximately a quarter of all patients with SJS‐TEN have involvement of their bronchial epithelium, and that up to half require mechanical ventilation during their acute admission.2 3 Duong et al. describe the long‐term follow‐up of a cohort of 32 patients who underwent objective testing of their respiratory function in the year following discharge from an acute admission with SJS, TEN or SJS/TEN overlap.1 They demonstrate that a significant number of such patients have abnormal pulmonary function tests, specifically diffusion capacities, noted at 3 months, with abnormalities sustained in a proportion of this cohort on retesting 6 months later. Pulmonary function test abnormalities were significantly associated with the extent of detachment of epidermis in the acute phase of the disease, allowing the practising clinician to risk stratify these patients. Of particular note is the fact that these patients were largely asymptomatic with respect to respiratory function; this is a salutary reminder to clinicians that while history taking at presentation may not reveal the true extent of disease, the extent of cutaneous manifestations may provide important clues to underlying pulmonary pathology. This should be actively investigated, as it may influence pulmonary function in the future.