title: Generalized erythrodermic pemphigus foliaceus in a child and its successful response to rituximab treatment.
abstract: Pemphigus foliaceus is an autoimmune disease that clinically manifests with cutaneous blisters of the superficial skin. The nonendemic or sporadic form of this entity is rare in children and typically presents with a milder, more localized rash that usually follows a benign course of short duration. We describe an affected patient atypical in both her young age and the severity of skin findings. Our patient presented with a full body exfoliative erythroderma at 21 months of age. After an extensive work-up to determine the etiology of her exfoliative erythroderma, direct and indirect immunofluorescence studies confirmed the diagnosis of pemphigus foliaceus. Rituximab therapy was initiated based on the patient's refractory disease course to multiple immunosuppressive agents. Rituximab is a therapeutic monoclonal antibody targeting CD20, an integral membrane protein highly expressed on the surface of pre-B lymphocytes and activated mature B lymphocytes. The patient's skin exhibited marked clinical improvement after the start of rituximab infusions over 12 weeks. Her initial desmoglein 1 antibody level was greater than 1:1280, which decreased to 1:16 after seven rituximab treatments. She has had no skin flares since initiating treatment with rituximab therapy. Based on this clinical and serologic response, the use of rituximab may be helpful in the treatment of pediatric pemphigus foliaceus refractory to mainstays of therapy.
authors.affiliation: Department of Dermatology and Cutaneous Surgery, Division of Pediatric Dermatology, University of Miami Miller School of Medicine, Miami, Florida 33125, USA.
authors.forename: Elizabeth Alvarez
authors.initials: EA
authors.lastname: Connelly
authors.forename: Cheryl
authors.initials: C
authors.lastname: Aber
authors.forename: Gary
authors.initials: G
authors.lastname: Kleiner
authors.forename: Carlos
authors.initials: C
authors.lastname: Nousari
authors.forename: Carlos
authors.initials: C
authors.lastname: Charles
authors.forename: Lawrence A
authors.initials: LA
authors.lastname: Schachner
mesh_terms: D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D003873:Dermatitis, Exfoliative / Q000188:drug therapy* / Q000209:etiology / Q000473:pathology; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D007223:Infant; D010392:Pemphigus / Q000150:complications / Q000188:drug therapy* / Q000473:pathology*; D000069283:Rituximab
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D000069283:Rituximab