Bibush Amatya, Aarif Syed MM and Leison Maharjan
Introduction: Pemphigus erythematosus, also known as the Senear-Usher syndrome, is an autoimmune condition where there is overlap between the clinical and immunological features of pemphigus foliaceous and lupus erythematosus. Patients present with erythematous, scaly, and crusted lesions in seborrhoeic distribution along with malar rash in butterfly distribution. Diagnosis is based on clinical findings, characteristic histopathologic changes, presence of antinuclear antibodies and immunolabelling in direct immunofluorescence test.
Case Presentation: We report a case of a middle-aged Nepali male who presented with the complaints of photosensitivity, itchy rash on the cheeks and flaccid blisters and crusted erosions on the chest for a few weeks. His diagnosis of Senear-Usher syndrome was based on clinical, histological and immunological findings. He was subsequently started on oral prednisolone and hydroxychloroquine sulfate, along with topical corticosteroids, which induced remission within the first week. He is currently on maintenance therapy without any relapse. This is to our knowledge, the first case of Senear-Usher syndrome reported from Nepal.
Conclusion: Topical and oral corticosteroids along with hydroxychloroquine sulfate should be considered first line in the treatment of Senear-Usher syndrome.