Background The multi-morbid burden and use of systemic immunosuppressants in people with psoriasis may confer greater risk of adverse COVID-19 outcomes but data are limited. Objective Characterize the course of COVID-19 in psoriasis and identify factors associated with hospitalization. Methods Clinicians reported psoriasis patients with confirmed/suspected COVID-19 via an international registry, PsoProtect. Multiple logistic regression assessed the association between clinical/demographic characteristics and hospitalization. A separate patient-facing registry characterized risk-mitigating behaviours. Results Of 374 clinician-reported patients from 25 countries, 71% were receiving a biologic, 18% a non-biologic and 10% no systemic treatment for psoriasis. 348 (93%) fully recovered from COVID-19, 77 (21%) were hospitalized and nine (2%) died. Increased hospitalization risk was associated with older age (multivariable-adjusted OR 1.59 per 10 years, 95% CI 1.19-2.13), male sex (OR 2.51, 95% CI 1.23-5.12), non-white ethnicity (OR 3.15, 95% CI 1.24-8.03) and comorbid chronic lung disease (OR 3.87, 95% CI 1.52-9.83). Hospitalization was more frequent in patients using non-biologic systemic therapy than biologics (OR 2.84, 95% CI 1.31-6.18). No significant differences were found between biologic classes. Independent patient-reported data (n=1,626 across 48 countries) suggested lower levels of social isolation in individuals receiving non-biologic systemic therapy compared to biologics (OR 0.68, 95% CI 0.50-0.94). Conclusion In this international moderate-severe psoriasis case series, biologics use was associated with lower risk of COVID-19-related hospitalization than non-biologic systemic therapies, however further investigation is warranted due to potential selection bias and unmeasured confounding. Established risk factors (being older, male, non-white ethnicity, comorbidities) were associated with higher hospitalization rates. Clinical Implications We identify risk factors for COVID-19-related hospitalization in psoriasis patients, including older age, male sex, non-white ethnicity and comorbidities. Use of biologics was associated with lower hospitalization risk than non-biologic systemic therapies.
This series is the largest reported to date and highlights that individuals with different forms of epidermolysis bullosa are at risk of developing squamous cell carcinomas of the skin. Patients often develop multiple primary tumours that tend to behave aggressively despite wide local excision and have poor response to conventional chemotherapy. The prognosis in severe recessive dystrophic epidermolysis bullosa is worse than previously reported, with median survival from diagnosis of first squamous cell carcinoma of only 2.4 years.Epidermolysis bullosa (EB), notably severe recessive dystrophic EB (RDEB-S), is associated with increased risk of aggressive mucocutaneous squamous cell carcinomas, the major cause of mortality in early adulthood. This observational, retrospective case review describes a series of EB patients with cutaneous squamous cell carcinomas over a 28-year period. Forty-four EB patients with squamous cell carcinomas were identified with a total of 221 primary tumours. They comprised: 31 (70%) with RDEB-S, 4 (9%) with other RDEB subtypes, 5 (11.4%) with dominant dystrophic EB, 3 (6.8%) with intermediate junctional EB and 1 (2.3%) with Kindler EB. Squamous cell carcinomas occurred earlier in RDEB-S (median age 29.5 years; age range 13-52 years) than other groups collectively (median age 47.1 years; age range 30-89 years) and most had multiple tumours (mean 5.8; range 1-44). Squamous cell carcinoma-associated mortality was high in RDEB-S (64.5%), with median survival after first squamous cell carcinoma of 2.4 years (range 0.5-12.6 years), significantly lower than previous reports, highlighting the need for early surveillance and better treatments.
al. Comparison of coronary computed tomography angiography, fractional flow reserve, and perfusion imaging for ischemia diagnosis. J Am Coll Cardiol 2019;73: 161e73.
ObjectivesThe aims of the authors’ case series were to outline the clinical features of prepubertal nocturnal vulval pain syndrome and to look at management and outcomes.MethodsClinical details of prepubertal girls experiencing episodes of nocturnal vulval pain with no identifiable cause were recorded and analyzed. Parents completed a questionnaire to look at outcomes.ResultsEight girls with age at onset of symptoms between 3.5 and 8 years (mean 4.4 years) were included. Each patient described intermittent episodes of vulval pain lasting between 20 minutes and 5 hours, starting 1–4 hours after falling asleep. They were crying and rubbing or holding the vulva with no obvious cause seen. Many were not fully awake and 75% had no recollection of the events. Management focused on reassurance alone. The questionnaire showed that 83% had full resolution of symptoms with a mean duration of 5.7 years.ConclusionsPrepubertal nocturnal vulval pain syndrome may be a subset of vulvodynia (generalized, spontaneous, intermittent) to be included in the clinical spectrum of night terrors. Recognizing the clinical key features should aid prompt diagnosis and reassurance of the parents.
Hidradenitis suppurativa (HS) is a chronic inflammatory disease characterized by recurrent painful nodules, abscesses and sinus tracts in predominantly apocrine gland-bearing skin. Adalimumab is an antitumour necrosis factor biologic approved by the National Institute for Health and Care Excellence in June 2016 for the management of moderate-to-severe HS and is the only licensed biologic for HS. We conducted a retrospective review of patients attending our specialist HS service who were initiated on adalimumab. Demographics, Hurley stage, HS physician global assessment (HS-PGA), patient-reported psychosocial outcomes and reasons for treatment discontinuation were examined. The cohort comprised 57% females (53%). Seventy-four per cent of patients were overweight or obese (n = 80), with a mean weight of 90 kg. Seventy-eight per cent were current or ex-smokers (n = 83). Twelve per cent reported a positive family history of HS (n = 13). Patients had an average baseline Hurley score of 2.8, representing moderate-to-severe disease (Hurley stages 2–3) and a HS-PGA of 3.7 (3 = moderate and 4 = severe). Concomitant treatment with antibiotics and/or systemic therapy was required in 44% of patients (n = 48). Oral antibiotics (n = 30; 28%), Septrin® (n = 16; 15%) and acitretin (n = 10; 9%) were the three most commonly coprescribed medicines. Nineteen per cent of patients underwent a surgical procedure for HS during treatment. The average treatment duration of adalimumab was 22 months. Thirty-two per cent of patients discontinued. Reason(s) for discontinuation included primary failure (n = 13), side-effects (n = 12), malignancy (n = 3; lung adenocarcinoma, plasma cell myeloma, extramammary Paget disease), COVID-19 concerns (n = 3), loss to follow-up (n = 2), secondary failure (n = 2), personal stress (n = 1), serious infection (n = 1; tuberculosis) and pregnancy (n = 1). Statistically significant improvements in HS-PGA score at 16 weeks were demonstrated compared with baseline (3.7 vs. 2.8; P < 0.001). Hurley score remained stable at these timepoints. Disease-related quality of life (Dermatology Life Quality Index) also significantly improved from 18.35 at baseline (reflecting severe impact) to 12.4 at 16 weeks (P < 0.001). Statistically significant differences were also found for the following patient-reported outcomes at 16 weeks compared with baseline (rated out of 100): severity (67 vs. 52; P < 0.013), odour (59 vs. 49; P = 0.008), discharge (64 vs. 46; P < 0.001), pain (65 vs. 46; P < 0.001) and fatigue (66 vs. 54; P = 0.006). Paired t-tests were used for all statistical analyses. In summary, adalimumab is an effective treatment for moderate-to-severe HS leading to clinical improvement in the HS-PGA scale and patient-reported outcomes. One-third of patients discontinued treatment due to a lack of response or side-effects. Adalimumab is often used in combination with other systemic therapies and surgery, reflecting the challenges of HS management.
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