011 Investigating the role of skin-resident T cells in pemphigus vulgaris in humans

Abstract

Pemphigus is a chronic bullous disease with a poor prognosis, especially in case of a mucocutaneous involvement. Due to immune disturbance and skin barrier deficiency, complications are frequent. We performed a retrospective cohort study based on medical records of 303 hospitalized pemphigus patients in the Dermatology department of Ibn Sina University Hospital between 1990-2020. Data collected include: age, history, mean duration of disease before consultation, bedridden duration, clinical phenotype, death. The mean age was 53 year-old, average duration of disease was 13.5 months, bedridden duration was 75 days, steroids were used in 55%, steroids + azathioprine in 45%. Among 303 patients: 42,5% developed complications, they were distributed as follow: infectious 75.4% (50 candidiasis, 36 herpes-simplex, 10 pseudomonas aeuroginosa, 1 varicella-zoster, 7 Covid, 4 erysipelas, 20 patients died due to a severe septic shock), vascular 25.4% (9 venous thrombosis, 8 high blood pressure, 4 acute ischemic stroke), osteoarticular 18.2% (20 osteoporosis), endocrine dysfunction in 17.4% (12 diabetes, 10 Cushing syndrome), psychiatric and ocular complications in 8.7% each: mainly 10 depression, 7 cataract and 3 glaucoma. Patients with pemphigus vulgaris were more likely to develop complications (59 versus 35 pemphigus erythematosus). The risk of complications was closely related to pre-existing comorbidities: 10 patients with diabetes, 10 tuberculosis, 6 cardiovascular disease, 6 thyroid dysfunction, 2 atopic dermatitis, 1 hidradranite suppurativa, 6 neoplasms (1 ovary cancer, 1 breast cancer, 2 myelodysplasia, 1 papillary thyroid cancer, 1 squamous-cell carcinoma). Our study demonstrated the role of anterior comorbidities, the late time of consultation and the long-term use of steroids in the promotion of various complications. A special care provided by a multidisciplinary team is therefore required to prolong survival and improve the quality of life.