Pediatric autoimmune blistering disorders - a five-year demographic profile and therapy experience.

Abstract

There is a paucity of literature on pediatric autoimmune bullous disorders (AIBD) in the Indian population. We aimed to retrospectively evaluate the clinico-demographic profile of pediatric AIBDs in our patients and their response to various therapeutic modalities. This was a retrospective chart review of patients enrolled in our immunobullous disease clinic from November 2013 to August 2019. The clinical records of all the patients aged less than 18 years old with a definitive diagnosis of AIBD were reviewed based on clinical, histopathological, and immunological features. Forty out of 1209 patients with AIBD (3.3%) belonged to the pediatric age group. Pemphigus vulgaris (PV) was the most common AIBD (24, 60%) followed by chronic bullous disease of childhood (CBDC) at 15% (6) and pemphigus foliaceus (PV) at 12.5% (5). Subepidermal blistering disorders had a significantly younger age of onset (P = 0.04) compared to intraepidermal blistering disorders but higher frequency of achieving complete remission off therapy (P = 0.02). The mean time to achieve remission was significantly shorter in PV patients treated with a combination of rituximab and corticosteroids compared to those treated with oral prednisolone and oral immunosuppressive adjuvants (P = 0.001). Rituximab was tolerated well in all 12 pemphigus patients. Oral lesions in PV patients took significantly longer time to achieve remission compared to the cutaneous lesions (P = 0.001). PV was the most common pediatric AIBD in Indian patients. Rituximab was a safe and effective modality of treatment in moderate to severe pediatric pemphigus.