Drug‐resistant bullous pemphigoid and inflammatory bowel disease in a pediatric case successfully treated by plasma exchange and extracorporeal photochemotherapy

Abstract

Bullous pemphigoid (BP) is an autoimmune skin disease that occurs mainly in elderly patients; onset of BP is rare in childhood. Inflammatory bowel diseases (IBD), by contrast, have a pediatric onset in 25% of presenting cases, requiring expert multidisciplinary management. Here we report a pediatric case of IBD (involving stomach, duodenum, ileum, and colon-rectum) associated with a disseminated form of drug-resistant BP successfully treated by plasma exchange (PEX), extracorporeal photochemotherapy (ECP), and corticosteroid therapy. The addition of PEX and ECP to standard treatment induced no severe side effects, prompted a rapidly achieved complete and long-term remission, and allowed dose tapering of the immunosuppressive drugs over an 18-month follow-up.

British Association of Dermatologists’ guidelines for the management of bullous pemphigoid 2012

“…29 Other small case series and anecdotal case reports have used different regimens of treatment with varying outcomes. [105][106][107] A child with BP and inflammatory bowel disease was treated with plasma exchange in combination with extracorporeal photochemotherapy that allowed reduction of immunosuppressive agents. 105 Immunoapheresis is a procedure by which immunoglobulins are removed from the circulation without the need to replace the plasma.…”

“…[105][106][107] A child with BP and inflammatory bowel disease was treated with plasma exchange in combination with extracorporeal photochemotherapy that allowed reduction of immunosuppressive agents. 105 Immunoapheresis is a procedure by which immunoglobulins are removed from the circulation without the need to replace the plasma. Only a few case reports [108][109][110] describe immunoapheresis as an adjuvant treatment for BP, allowing remission with a reduced dose of systemic steroids.…”

Juvenile bullous pemphigoid: the presentation and follow‐up of six cases

“…The age ranged from 2.5 months to 14 years, with 15 cases at the age of 1 year or younger and 41 cases younger than 8 years. To the best of our knowledge, up to November 2008, there were 28 additional cases, giving 81 as the total number of published cases of JBP 5–16 . The disease most often occurs in children older than 8 years.…”

“…To the best of our knowledge, up to November 2008, there were 28 additional cases, giving 81 as the total number of published cases of JBP. [5][6][7][8][9][10][11][12][13][14][15][16] The disease most often occurs in children older than 8 years. The average age at JBP onset in our study was 10 years, with a female to male ratio of 2 : 1.…”

Severe Bullous Pemphigoid in an Infant— Successful Treatment with Rituximab

“…Only two children received additional dapsone (4) or mycophenolate mofetil (1), respectively. There are other reports of successes with cyclosporin, azathioprine, and plasmapheresis (4–6).…”