Background Remission duration and treatment response following phototherapy for psoriasis are highly variable and factors influencing these are poorly understood. Objectives Our primary outcome was to investigate whether selected clinical/serum biomarkers were associated with remission duration, and secondly with psoriasis clearance at the end of phototherapy. In addition, we looked at whether early trajectory of UVB clearance was associated with final clearance outcome. MethodsWe performed a prospective cohort study of 100 psoriasis patients, routinely prescribed Narrowband UVB and measured selected clinical and biochemical biomarkers, including weekly PASI (psoriasis area and severity index) scores. Patients were followed up for 18 months. ResultsThe median time to relapse was 6 months (95% CI 5-18) if PASI90 was achieved, and 4 months (95% CI 3-9) if less than PASI90 was achieved. Achieving PASI100 did not result in prolonged remission. On UVB completion, the median final PASI (n = 96) was 1.0 (IQR 0.5, 1.6) with 78 (81%) achieving PASI75 and 39 (41%) achieving PASI90.Improved PASI90 response was significantly associated with lower BMI, higher baseline PASI, non-smoking status and lower cumulative NbUVB. Serum levels of C-reactive protein (CRP) and vitamin D were not associated with clearance or remission duration. Early treatment response from weeks 2-3 was predictive of final outcome. For example, achieving PASI30 at week 3 was significantly associated with PASI90 at the end of the course [36/77 (51%) vs. 2/24 (8%), P < 0.001].Conclusions Raised BMI and positive smoking status predicted poorer phototherapy response. For the first time, we have shown that PASI clearance trajectory over the first 2-3 weeks of UVB, can predict psoriasis clearance. This is an important new step towards developing psoriasis personalized prescribing, which can now be formally tested in clinical trials. These simple clinical measures can be used to inform patient treatment expectations; allowing treatment modifications and/or switching to alternative therapies.
Background Autoimmune bullous disorders, encompassing pemphigus and pemphigoid diseases, are associated with significant morbidity and mortality. This is in part due to high cumulative doses of corticosteroids in combination with immunosuppressant agents used in traditional treatment regimes. Rituximab is an antiCD20 monoclonal antibody which can induce complete remission, but it is currently unlicensed in the UK and approved only after other treatments have failed. Methods We report a retrospective cohort study of 33 patients with pemphigus and pemphigoid diseases treated with rituximab from a single tertiary centre from 2013 to 2019. Results “Complete remission off therapy” was achieved by 27.3% (n = 9), and a further 27.3% (n = 9) had complete remission on minimal therapy. Twenty‐one per cent (n = 7) had “partial remission on minimal therapy”; 9.1% (n = 3) patients were in the “consolidation phase,” and 12.1% (n = 4) had a “relapse/flare.” A steady reduction in prednisolone doses was observed post‐Rituximab infusion. Pre‐Rituximab the median dose of prednisolone was 20mg (range 10‐35, IQR 25), 15mg (range 9.5‐22.5, IQR 13) at 1 month, 9mg (range 5‐10, IQR 5) at 6 months, 4mg (range 0‐5mg, IQR 5) at 12 months and 0 (0‐4.35, IQR 4.25) at 18 months. Twelve per cent (n = 4) of patients had documented infusion reaction symptoms. Twelve per cent (n = 4) had later infective complications. Conclusion This real clinic data adds to the evidence that Rituximab is a safe and effective treatment for both pemphigus and pemphigoid autoimmune blistering conditions. Significantly, we were able to demonstrate a substantial reduction in corticosteroid dosage in our cohort of patients following rituximab treatment.
A 59-year-old man was referred to an urgent skin cancer dermatology clinic with a 4-month history of multiple skin lesions on his trunk, arms and face. The lesions were painful, increasing in size, bleeding and oozing serous fluid. The patient reported being otherwise systemically well, with a history of mild eczema and a background of high alcohol intake. On examination, he had a striking rash with numerous large rupioid plaques with thick crusting and surrounding erythema on the trunk and limbs. Smaller plaques were seen on the face and neck. An infective aetiology was favoured with an initial differential diagnosis of ecthyma or atypical mycobacterial infection. A biopsy was taken, as well as screening blood tests. Treponemal antibodies were detected; rapid plasma reagin was reactive (1 : 32), as was Serodia Treponema pallidum particle agglutination test (1 : 1280), suggesting active syphilis infection. His biopsy demonstrated granulomatous inflammation, associated with neutrophils, plasma cells and increased mucin deposition. Immunohistochemical staining for T. pallidum confirmed corkscrew-like organisms within the basal epidermis and upper dermis, consistent with secondary syphilis; screening for concurrent HIV infection was negative. The patient was referred to a local genitourinary clinic for treatment with benzylpenicillin and for full sexual health screening. The word ‘rupioid’ describes oyster or limpet shell-shaped thick keratotic lesions and is derived from the Greek word rhupos, meaning ‘filth’. Differential causes for rupioid lesions include cutaneous T-cell lymphoma, psoriasis, scabies and syphilis. Rupioid syphilis is also termed ‘malignant syphilis’ or ‘Lues maligna’, describing well-demarcated plaques with thick, lamellate and adherent crusts. It is most frequently encountered in HIV-positive patients; in HIV-negative patients, the associated comorbidities are diabetes, alcoholism, drug abuse, psoriasis and hepatitis. Typically, there is rapid clinical improvement once antibiotic treatment is administered, but episodes of Jarisch Herxheimer reaction are reported. Syphilis diagnoses have tripled over the last 10 years, with cases in the heterosexual population rising most rapidly. Dermatologists have a pivotal role to play in diagnosing primary and secondary syphilis, thereby preventing the long-term sequelae of tertiary syphilis and controlling the rising prevalence of the disease in the general population. This case highlights a striking and rare manifestation of secondary syphilis which, once seen, will not be forgotten.
Toxic epidermal necrolysis (TEN) is associated with high mortality and morbidity. Owing to a lack of robust evidence, treatment guidelines are ambiguous and clinicians must use discretion in management decisions, specifically with regard to the use and choice of systemic agents, including immunosuppressants. We present a retrospective study of cases referred to a tertiary referral centre between 2015 and 2022. Cases were identified using histopathology coding and a database compiled by clinicians. Forty cases were categorized as follows: TEN (n = 12); Stevens–Johnson syndrome (SJS)/TEN overlap (n = 5); SJS (n = 5); unclear (n = 9); insufficient information (n = 5) and others [n = 9 (lupus, n = 4; erythema multiforme major, n = 3; burn, n = 1; recurrent erythema multiforme, n = 1)]. The median age of patients with TEN was 66 [interquartile range (IQR) 53.3–74; 33% men, 67% women]. The median percentage body surface area of epidermal loss was 40% (n = 8; IQR 30–70). Forty-two per cent of patients had an underlying malignancy. Implicated medications included nivolumab/ipilimumab (n = 2), co-trimoxazole (n = 2), carbamazepine (n = 1) and proton pump inhibitors (n = 3). Sixty-seven per cent of patients with TEN were managed by the burns centre, with a further 17% admitted to the intensive care unit. One patient presented directly to the dermatology team and 58% were transferred from other hospitals. The median time from the documented onset of rash to transfer to specialist care was 5 days (IQR 2.8–11.5). Thirty-three per cent of patients were given immunosuppression (intravenous methylprednisolone). The median duration of admission was 14 days (IQR 7.5–21.5). Fifty-eight per cent of patients (n = 7) survived. None of the five patients who died had received immunosuppression. The median time from onset of rash to receiving specialist care was 3.5 days for those who survived (n = 4) vs. 10 days for those who did not (n = 3; P = 0.03). Thirty-three per cent of patients had documented chronic complications (severe ophthalmic sequelae, n = 2; circumcision for phimosis, n = 1). This study was limited by the retrospective collection of data and the method used to identify cases. The current evidence for the treatment of TEN is primarily derived from small case series that often combine heterogeneous cases of TEN, SJS/TEN overlap and SJS. This is highly problematic as the risk–benefit dynamic varies greatly between patients with SJS, who have an excellent prognosis without treatment, and patients with TEN with a high associated mortality, who potentially have more to gain from immunosuppression. Our data support the need for patients to access specialist care promptly, to allow for early consideration of immunosuppression. Future research should focus on effective treatments for reducing mortality in cases of TEN specifically.
A previously well 16-year-old male presented with florid and widespread tense bullae and erosions. He gave a 2-week history of an erythematous rash consisting of crusted erosions that started on his chest and face. He denied any new medications or over-the-counter preparations. There was no preceding systemic illness, and no history of cold sores or sexual contacts. In the 24 h prior to his admission, his rash had become widespread, involving his trunk, limbs, hands, ears, face, lips, oral and genital mucosa. He had started to feel systemically unwell and was in significant pain. On examination, he had a combination of heavily crusted erosions and extensive tense bullae containing straw-coloured fluid on a background of inflamed skin. The morphology was herpetiform in some areas. There was notable extension of bullae onto the palms and penis, as well as intraoral and labial erosions. Viral skin swabs were negative, and his biopsy subsequently demonstrated subepidermal blistering with neutrophil-rich inflammation and positive direct immunofluorescence (IMF) for C3 and IgG only. Serum autoantibodies were negative. Childhood bullous pemphigoid (BP) was initially the working diagnosis, but indirect IMF showed localization to the base of the blister and anticollagen VII antibodies later proved positive, consistent with a diagnosis of epidermolysis bullosa acquisita (EBA). Given the clinical features, it was classified as BP-like EBA. He was initially treated with high-dose oral prednisolone, superpotent topical steroids and analgesia as an inpatient. Mycophenolate mofetil (MMF) was initiated, and his corticosteroid dose was slowly tapered. His condition was completely controlled with MMF 500 mg twice daily at 4 months. The patient then elected to stop taking MMF and remains in complete remission at 7 months. The EBA is rare in adults and even more so in children. The mean age of onset is 44–54 years, but up to 5% of reported cases are in children/adolescents. Four subtypes are described: classic mechanobullous, BP-like, mucous membrane pemphigoid-like and linear IgA bullous dermatosis-like. The BP-like EBA is characterized by widespread vesicles and tense bullae, commonly on an inflammatory background and lacking skin fragility, scarring or milia. Oral and genital mucosae are frequently affected in paediatric cases of EBA. Various steroid-sparing treatment modalities are described, including dapsone, colchicine and MMF. The prognosis in the paediatric population is favourable compared with that of adults: the majority achieve complete remission or complete control on treatment.
A previously well 16-year-old male presented with florid and widespread tense bullae and erosions. He gave a 2-week history of an erythematous rash consisting of crusted erosions that started on his chest and face. He denied any new medications or over-the-counter preparations. There was no preceding systemic illness, and no history of cold sores or sexual contacts. In the 24 h prior to his admission, his rash had become widespread, involving his trunk, limbs, hands, ears, face, lips, oral and genital mucosa. He had started to feel systemically unwell and was in significant pain. On examination, he had a combination of heavily crusted erosions and extensive tense bullae containing straw-coloured fluid on a background of inflamed skin. The morphology was herpetiform in some areas. There was notable extension of bullae onto the palms and penis, as well as intraoral and labial erosions. Viral skin swabs were negative, and his biopsy subsequently demonstrated subepidermal blistering with neutrophil-rich inflammation and positive direct immunofluorescence (IMF) for C3 and IgG only. Serum autoantibodies were negative. Childhood bullous pemphigoid (BP) was initially the working diagnosis, but indirect IMF showed localization to the base of the blister and anticollagen VII antibodies later proved positive, consistent with a diagnosis of epidermolysis bullosa acquisita (EBA). Given the clinical features, it was classified as BP-like EBA. He was initially treated with high-dose oral prednisolone, superpotent topical steroids and analgesia as an inpatient. Mycophenolate mofetil (MMF) was initiated and his corticosteroid dose was slowly tapered. His condition was completely controlled with MMF 500 mg twice daily at 4 months. The patient then elected to stop taking MMF and remains in complete remission at 7 months. EBA is rare in adults and even more so in children. The mean age of onset is 44–54 years, but up to 5% of reported cases are in children/adolescents. Four subtypes are described: classic mechanobullous, BP-like, mucous membrane pemphigoid-like and linear IgA bullous dermatosis-like. BP-like EBA is characterized by widespread vesicles and tense bullae, commonly on an inflammatory background and lacking skin fragility, scarring or milia. Oral and genital mucosae are frequently affected in paediatric cases of EBA. Various steroid-sparing treatment modalities are described, including dapsone, colchicine and MMF. The prognosis in the paediatric population is favourable compared with that of adults: the majority achieve complete remission or complete control on treatment.
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