Diagnosis and management of pemphigus: Recommendations of an international panel of experts

Steroid pulse therapy has shown satisfactory efficacy and safety in treating pemphigus vulgaris (PV). However, there is a paucity of data about the efficacy and safety of methylprednisolone, despite its frequent administration. The aim of this study is to evaluate the efficacy and safety of steroid pulse therapy in treating PV. In this 10‐year retrospective cohort study, 312 patients with PV, who had received methylprednisolone pulse therapy, were included. Data of pulse therapy sessions, adjuvant medications, dosages, remission rates, complications, and mortalities were collected from all patients. A total of 276 patients out of 312 underwent the final follow‐up at least 6 months after the last session of pulse therapy. Complete remission off therapy was achieved in 83 patients (30%), and 152 patients (55%) had complete remission on therapy. About 29 (10.5%) patients had lesions of pemphigus at the time of the study follow‐up, and 26.8% of remained patients were on the minimal therapy. Methylprednisolone pulse therapy could be considered as an option for proper control of PV in severe cases. It might lead to shorter periods of hospitalization and reduce the need to take long‐term high‐dose oral steroid therapy.

Section: Discussionmentioning

confidence: 99%

Methylprednisolone pulse therapy plus adjuvant therapy for pemphigus vulgaris: an analysis of 10 years' experience on 312 patients

Gheisari

Faraji

Dadras

et al. 2019

“…Several reports have demonstrated that rituximab therapy is effective for severe and refractory pemphigus (Ahmed & Shetty, ; Joly et al, ). Recent articles recommend rituximab for pemphigus, although relapse is common (Murrell et al, ). Thus, we investigated the clinical risk factors for poor clinical outcomes and relapse in pemphigus.…”

Section: Introductionmentioning

confidence: 99%

Mucosal involvement is a risk factor for poor clinical outcomes and relapse in patients with pemphigus treated with rituximab

Cho

Kim

Lim

et al. 2019

Many studies have reported the outcome of rituximab use in pemphigus but studies regarding the clinical risk factors for poor clinical outcomes or relapse are lacking. To clarify the risk factors for poor clinical outcomes or relapse in patients with pemphigus treated with rituximab, a retrospective chart analysis was performed on patients with pemphigus who were treated with rituximab in the dermatology clinic of Seoul National University Hospital. Forty patients with pemphigus were treated with rituximab, of which 39 (97.5%) experienced remission and 19 (48.7%) experienced relapse. Patients with mucosal lesions demonstrated poor clinical outcomes. The risk for relapse was 4.626 (confidence interval: 1.126–19.001, p = .034) times higher in patients with mucosal lesions than in those without lesions. In patients with pemphigus treated with rituximab, the presence of mucosal lesions resulted in poor clinical outcomes and frequent recurrence.

“…It should be noted that also cyclophosmamide, another common therapeutic option for pemphigus, has been associated with an increased risk of malignancies (Bernatsky et al, 2014). These considerations support the use of RTX as first-line therapy for moderate-to-severe pemphigus, as recommended by a recent consensus statement (Murrell et al, 2018), since its use has not been associated with a malignancy risk (van Daalen et al, 2017).…”

Section: Discussionmentioning

confidence: 89%

Rituximab as a dual therapeutic option for pemphigus and primary cutaneous B‐cell lymphomas: Two case reports

Michelerio

Novario

Barruscotti

et al. 2019

It is known that individuals with immune dysregulation have an increased risk of non‐Hodgkin lymphoma. This association has been proven for pemphigus as well as for other autoimmune disease. We describe the development of cutaneous B‐cell lymphoma in two patients affected by long‐standing pemphigus vulgaris and pemphigus foliaceus (i.e., characterized by histological and immunopathological features different from those of paraneoplastic pemphigus). In both cases, a therapy with rituximab allowed to achieve the complete remission for the lymphoproliferative disease (never recurred at follow up) and a substantial long‐term improvement of the clinical manifestations of pemphigus, although persistent to serological disease and occasional recurrences. We suggest that clinicians should consider that patients with long‐standing pemphigus, both vulgaris and foliaceus, may develop primary cutaneous B‐cell lymphomas, as shown in our report, and in these cases the treatment with rituximab is elective, providing a therapeutic option for both low‐grade or follicular, CD20‐positive, B‐cell non‐Hodgkin lymphomas and pemphigus. Nevertheless, as shown in our cases, a constant surveillance for pemphigus is necessary.