A single cycle of rituximab is an effective treatment for pemphigus. Because of its potentially severe side effects, its use should be limited to the most severe types of the disease. (ClinicalTrials.gov number, NCT00213512 [ClinicalTrials.gov].).

The cytokines controlling the development of human interleukin (IL) 17–producing T helper cells in vitro have been difficult to identify. We addressed the question of the development of human IL-17–producing T helper cells in vivo by quantifying the production and secretion of IL-17 by fresh T cells ex vivo, and by T cell blasts expanded in vitro from patients with particular genetic traits affecting transforming growth factor (TGF) β, IL-1, IL-6, or IL-23 responses. Activating mutations in TGFB1, TGFBR1, and TGFBR2 (Camurati-Engelmann disease and Marfan-like syndromes) and loss-of-function mutations in IRAK4 and MYD88 (Mendelian predisposition to pyogenic bacterial infections) had no detectable impact. In contrast, dominant-negative mutations in STAT3 (autosomal-dominant hyperimmunoglobulin E syndrome) and, to a lesser extent, null mutations in IL12B and IL12RB1 (Mendelian susceptibility to mycobacterial diseases) impaired the development of IL-17–producing T cells. These data suggest that IL-12Rβ1– and STAT-3–dependent signals play a key role in the differentiation and/or expansion of human IL-17–producing T cell populations in vivo.

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To assess the predictive value of antidesmoglein (Dsg) 1 and anti-Dsg3 antibody (Ab) enzymelinked immunosorbent assay (ELISA) values for the occurrence of relapses in pemphigus. Design: Retrospective study. Setting: Dermatology departments from 13 university hospitals in France. Patients: The study population comprised 26 patients with typical clinical, histologic, and immunofluorescence findings of pemphigus, who were followed up over a 17-month period. Main Outcome Measures: Serial anti-Dsg1 and anti-Dsg3 Ab ELISA values were recorded during the patients' follow-up examinations and correlated with the occurrence of skin and/or mucosal relapses. Results: A significant reduction of anti-Dsg1 (PϽ.001) and anti-Dsg3 (P <.001) Ab ELISA values was observed in serum samples from patients with pemphigus foliaceus or pemphigus vulgaris after the initial treatment. Dur