Introduction Cutaneous manifestations are central to the primary diagnosis of systemic lupus erythematosus (SLE). However, information on the clinical, histopathologic, and direct immunofluorescence (DIF) features among subtypes of cutaneous lupus erythematosus (CLE), as well as longitudinal prospective observational study to evaluate the natural history and the progression to SLE, is lacking among Asians. Our objectives are to summarize the differences in the clinical, histopathologic, and DIF characteristics and serological profiles between various subtypes of CLE, and to provide its natural history and the association with disease activity in our Asian population. Methods A prospective observational study on CLE patients was performed between May 2016 and May 2020. Patients underwent full physical/dermatologic examination, skin biopsy for histology, and DIF. Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) scores and laboratory data were evaluated. Time schedule and characteristics for resolution and/or the disease progression to SLE were recorded in subsequent follow-ups. Results Of 101 biopsy-proven CLE patients, 25 had acute CLE (ACLE), 8 had subacute CLE (SCLE), 39 had chronic CLE (CCLE) only, 22 had CCLE with SLE, and 7 had LE-nonspecific cutaneous lesions only. Patients with exclusive CLE showed lower female preponderance, serological abnormalities, and correlation to systemic disease. However, when CLE was accompanied with any LE-nonspecific cutaneous manifestations, they were associated with high antinuclear antibody (ANA) titer, renal, hematologic, joint involvement, and greater SLEDAI score. Of 207 biopsy sections, SCLE/CCLE regardless of systemic involvement showed significantly higher percentage of superficial/deep perivascular and perieccrine infiltration than ACLE. On DIF, deposition of multiple immunoreactants was associated with higher systemic disease. Approximately 10% of CLE-only patients later developed SLE but had mild systemic involvement. Conclusion Our findings support that each CLE subtype has a diverse and unique character. Comprehensive understanding of the differences among CLE subtypes is important for achieving the correct diagnosis and providing appropriate disease monitoring and management. Supplementary Information The online version contains supplementary material available at 10.1007/s13555-020-00471-y.

This is the largest single series of patients with SJS/TEN-like lupus erythematosus. Skin damage is an indicator of disease activity, and careful search for extracutaneous involvement to prevent further complications is mandatory.

Background: Rituximab provides more effective and less adverse effects than the standard dose of corticosteroids, but evidence on its efficacy and safety in the Thai population is lacking. Objective: To evaluate the efficacy and safety of rituximab in the treatment of pemphigus and also to determine prognostic factors linked to the treatment outcomes. Methods: Pemphigus patients who received rituximab from November 2017 to December 2020 were retrospectively reviewed. The outcome was evaluated by using early (end of consolidation phase [ECP]) and late endpoints (complete remission [CR] on/off therapy, immunological remission [IR], and relapse). Adverse events were noted. Prognostic factors associated with remission and relapse were analyzed. Results: Of 53 pemphigus patients, all attained ECP within 1.61 months. Almost 80% achieved CR on therapy within a median time of 6.36 months, while 33.9% reached CR off therapy in 19.74 months. Nearly half had IR within a median time of 6.88 months. Relapse occurred in 33.3% with a median time of 14 months. In multivariate analysis, receiving rituximab within 12 months of disease duration was more likely to achieve CR off therapy and IR (hazard ratio [HR] 3.79; 95% confidence interval [CI] 1.38, 10.42; P = 0.01 and HR 2.74; 95% CI 1.12, 6.69; P = 0.027, respectively), whereas older patients and positive antidesmoglein 1 levels at the time of CR were predictive indicators for relapse (HR 1.07; 95% CI 1.01, 1.13; P = 0.036 and HR 4.38; 95% CI 1.24, 15.46; P = 0.022, respectively). The treatment-related adverse effects occurred in 33.9%. Conclusion:Rituximab is effective and safe in Thai pemphigus patients. Early administration of rituximab was a predictor of clinical and immunological remission. Older age and persistently positive anti-Dsg1 were correlated with disease relapse.