Use of biologics in Chinese pregnant patients with deficiency of interleukin-36 receptor antagonist (DITRA): A case series.

OBJECTIVE: Impetigo herpetiformis (IH) is a rare form of pustular psoriasis which may result in maternal and fetal morbidity and even mortality. Deficiency of interleukin-36 receptor antagonist (DITRA) is the most frequently identified genetic defect of IH. Currently there are no biologics approved for IH despite the revolutionary role of biologics in the treatment of plaque and pustular psoriasis. Anecdotal reports of biologics use in DITRA patients with IH are also limited. CASE REPORTS: We present herein a case series of 6 Chinese IH patients harboring IL36RN gene c.115+6T>C mutation during 8 pregnancies, treated with various biologics, including adalimumab, etanercept and secukinumab. CONCLUSION: Most pregnancy courses were uneventful, except for one woman who had recurrent episodes of decreased fetal heart rate variability after adalimumab injections, which subsided after switching to etanercept. The treatment effectiveness and safety demonstrated in our cases suggested the role of biologics for the treatment of IH in patients with DITRA.

Clinical Effectiveness and Safety of Initial Combination Therapy with Corticosteroids and Rituximab in Bullous Pemphigoid: A Retrospective Cohort Study.

BACKGROUND: Rituximab is a potential initial adjuvant therapy for bullous pemphigoid, yet clinical experience is scarce. OBJECTIVE: We aimed to examine the clinical outcomes and safety of initial combination therapy with systemic corticosteroids and adjuvant rituximab for the treatment of bullous pemphigoid. METHODS: A retrospective cohort study was performed on 84 patients with bullous pemphigoid, who received systemic corticosteroids with or without initial adjuvant rituximab therapy (defined as rituximab use within 12 weeks after initiation of systemic corticosteroids). RESULTS: Among the 84 patients included (37 received systemic corticosteroids with rituximab and 47 were treated with systemic corticosteroids without rituximab), the median time to complete remission on minimal therapy or off therapy was 215 days (95% confidence interval 176.9-253.1) in patients receiving rituximab vs 529 days (95% confidence interval 338.6-719.4) in those not receiving rituximab. A Cox regression analysis showed an increased probability of reaching complete remission on minimal therapy or off therapy with the combined therapy (hazard ratio = 2.28 [1.28-4.07], p = 0.005) after age, Bullous Pemphigoid Disease Activity Index score, and underlying diseases were controlled. In multivariate logistic/linear regressions, initial adjuvant rituximab therapy was associated with a higher complete remission rate (odds ratio = 6.63 [2.09-21.03]) and lower cumulative prednisolone (mg)/body weight (kg) (B = -24.86 [-44.06 to -8.29]) within 48 weeks. Risk of hospitalization for infection was not elevated in the group treated with adjuvant rituximab. CONCLUSIONS: Rituximab use as adjuvant therapy within 12 weeks after initiation of systemic corticosteroids was associated with a faster and higher rate of achieving complete remission on minimal therapy or off therapy, as well as a significant corticosteroid-sparing effect and a comparable safety profile in this retrospective study. Hence, initial combination therapy with corticosteroids and adjuvant rituximab could serve as an effective treatment option for bullous pemphigoid, but this requires confirmation in randomized controlled studies.

A mask R-CNN based automatic assessment system for nail psoriasis severity.

Nail psoriasis significantly impacts the quality of life in patients with psoriasis, which affects approximately 2-3% of the population worldwide. Disease severity measures are essential in guiding treatment and evaluation of therapeutic efficacy. However, due to subsidy, convenience and low costs of health care in Taiwan, doctor usually needs to manage nearly hundreds of patients in single outpatient clinic, leading to difficulty in performing complex assessment tools. For instance, Nail Psoriasis Severity index (NAPSI) is used by dermatologists to measure the severity of nail psoriasis in clinical trials, but its calculation is quite time-consuming, which hampers its application in daily clinical practice. Therefore, we developed a simple, fast and automatic system for the assessment of nail psoriasis severity by constructing a standard photography capturing system combined with utilizing one of the deep learning architectures, mask R-CNN. This system not only assist doctors in capturing signs of disease and normal skin, but also able to extract features without pre-processing of image data. Expectantly, the system could help dermatologists make accurate diagnosis, assessment as well as provide precise treatment decision more efficiently.