Pemphigus foliaceus: Clinicoepidemiological study at tertiary care center

Abstract

Background: Pemphigus foliaceus (PF) is characterized by shallow erosions with erythema, scaling, and crusting over seborrheic areas of the face, scalp, and chest with characteristic histopathology and direct immunofluorescence (DIF). Aim: To study the clinicoepidemiological profile of patients with PF and response to treatment, especially pulse therapy. Materials and Methods: This was a prospective clinical and therapeutic study of 20 patients WITH PF, attending DVL Department, from May 2007 to April 2016. The diagnosis was made based on clinical features, histopathology, and DIF. Clinical variants of PF were studied, and severity was assessed based on body surface area involvement. After detailed evaluation, 18 patients started on pulse therapy [13 dexamethasone cyclophosphamide pulse (DCP), 3 dexamethasone azathioprine pulse (DAP), 2 dexamethasone-only pulse (DOP)]. Two patients were not started on pulse therapy for medical reasons. Results: The most common age group affected was 51–60 years. Male: female ratio was 1:1. Clinical variants observed were classical localized – 4, classical generalized – 10, erythrodermic – 4, pemphigus erythematosus – 1, and acral vesiculopustular lesions – 1. Nail changes and small erosion on the inner aspect of the lower lip were present in two patients each. The average time to remission was 7.3 months. Of 18 patients, 14 patients responded well to DCP/DAP/DOP therapy. Two patients lost to follow-up in phase 1. Two patients had frequent relapses and started on methotrexate; the disease is under control in one patient. Conclusion: PF usually localized to seborrheic areas; in our study, generalized papulosquamous lesions and erythroderma-like presentation were predominant. The majority of the patients showed remission with pulse therapy, and two patients require addition of immunosuppressants in the follow-up period.