**13. Pemfigus vulgaris in children**

PV is the most common type in the pediatric group among all pemphigus types, excluding endemic pemphigus foliaceus [43]. The pediatric variant is divided into two, childhood PV (0–12 years) and adolescent (juvenile) PV (13–18 years) [44]. Most patients have mucocutaneous involvement, both in childhood and in the juvenile form. Oral, nasal, ocular and anal mucous membranes are involved. The frequency of genital involvement is high in both groups [45]. The clinical picture is similar to that in adults. Loose bullae on intact or erythematous skin open easily and become eroded and crusted lesions. Antibody titrations can be detected in the serum in most of patients [43]. Nikolsky's sign is positive.

Systemic steroid as in adults, is the cornerstone of treatment [45]. Systemic side effects develop in two-thirds of patients treated with systemic steroids. The most common side effects are; Cushing's syndrome (65%), growth retardation (50%) and infection (50%). Prednisone dose is started at 40–60 mg/day, when new lesion growth stops, it is gradually reduced and adjuvant therapy is added. Many adjuvant agents such as AZA, dapsone, mycophenolate mofetil, cyclophosphamide, methotrexate, cyclosporine, plasmapheresis, IVIg and rituximab have been used in the treatment of children as in adults. Data on the use of rituximab therapy in pediatric patients are limited. The youngest case treated with rituximab was a 4-year-old girl [44]. There are partial and complete remission reports in the literature. There is no standard dosing regimen in children. The lymphoma protocol was used in most of the published cases. Treatments usually last 2–3 years and the prognosis is better than in adult patients [46].
