Rituximab in Childhood Pemphigus Vulgaris: A Long-Term Follow-Up Case and Review of the Literature

Abstract

Pemphigus vulgaris is an infrequent but life-threatening autoimmune blistering disease that is rare in the pediatric age. Although the mainstay of therapy for childhood pemphigus vulgaris (CPV) is steroids, adjuvant immunosuppressive drugs are often needed to control the disease. Thus, an important part of CPV morbidity can be related to treatment. We report the youngest CPV patient described in the English literature, an 18-month-old boy, who has been followed up for 16 years. During this period, the patient has received several immunosuppressive therapies with variable response. He has finally achieved a long-lasting and complete remission with rituximab. Successful treatment with rituximab has previously been reported in 6 cases of CPV, in whom rituximab presented a good side effect profile. Our patient has experienced a chronic and severe clinical course with multiple flares eventually developing vegetative lesions. Since he presented refractoriness to multiple therapies, we administered rituximab. The introduction of this drug led to a dramatic clinical response and a long-term clinical remission. Based on the experience of this case and the data reported in the literature, we believe that rituximab may be a safe and efficacious agent for the treatment of severe CPV.

Novel applications of Rituximab in dermatological disorders

“…Based on the experience and the data reported in the literature, it is believed that rituximab may be a safe and efficacious agent for the treatment of severe CPV. [45]…”

Pénfigo vulgar de la infancia, a propósito de un caso

“…2 En la infancia, es infrecuente; representa el 1,4%-2,9% de todos los casos de pénfigo vulgar. 3 La edad promedio de presentación son los 12 años y la relación hombremujer es 1:0,96. Esta forma de presentación es la más frecuente en la infancia, a excepción del pénfigo endémico en Brasil.…”

“…4,5 Actualmente, el rituximab, un anticuerpo monoclonal dirigido contra el antígeno CD20 de e460 / Arch Argent Pediatr 2016;114(6):e457-e460 / Presentación de casos clínicos los linfocitos B, ha sido propuesto para aquellos casos refractarios a los corticoides. 3,9,10 Es importante también tener en cuenta el control nutricional, la analgesia, la higiene y la curación de heridas. 6 …”

Juvenile Pemphigus Vulgaris

“…8 The mortality rate in children (2.9%) is lesser than in adults (10-15%) due to this reduced immunosuppression. 8,9 Based on the rationale that pemphigus is primarily an autoantibodydriven autoimmune disorder, therapies that deplete autoreactive B-cell clones have been investigated for the treatment of pemphigus. 10 Rituximab, a monoclonal antibody directed against the CD20 antigen on B lymphocytes, has demonstrated efficacy for PV and pemphigus foliaceus.…”