Dupilumab for atopic dermatitis in metastatic cancer.

We present a case of a 47-year-old male with severe atopic dermatitis and metastatic renal cell carcinoma managed successfully with dupilumab. This case further supports the safety of dupilumab in patients with active malignancy, an area currently with limited data.

Treatments for atopic dermatitis.

Atopic dermatitis usually develops in childhood, but can occur in adults. Management involves drug and non-drug treatments to clear the skin. Not all patients with atopic dermatitis have allergies. Most patients have trigger factors that can be avoided. All patients should use soap substitutes and bath oils. Moisturisers are important for improving the condition of the skin. Topical corticosteroids are the main drug treatment. The choice of corticosteroid depends largely on the site of the atopic dermatitis. Topical calcineurin inhibitors can be considered for sensitive sites such as the face where potent topical corticosteroids are potentially harmful. Adjunctive treatments given during flares of dermatitis include bleach baths and wet dressings. Antihistamines may help to relieve itch. Phototherapy may be considered by a specialist for adults if there is inadequate response to treatment. Severe cases of atopic dermatitis may require systemic treatment. Immunosuppressants, such as ciclosporin, have been used and now dupilumab and upadacitinib are available for severe chronic atopic dermatitis.

A real-world Australian experience of upadacitinib for the treatment of severe atopic dermatitis.

Upadacitinib is a selective Janus kinase-1 (JAK-1) inhibitor that has been shown in clinical trials to be effective for the treatment of moderate-to-severe atopic dermatitis (AD). This study aimed to evaluate the real-world experience of patients with AD treated with upadacitinib in a single-centre Australian cohort. Our study revealed a higher propensity for herpetic infections compared with previous randomised controlled trials (RCTs).

Efficacy and safety of givosiran for acute hepatic porphyria: Final results of the randomized phase III ENVISION trial.

BACKGROUND & AIMS: Acute hepatic porphyria (AHP) is caused by defects in hepatic heme biosynthesis, leading to disabling acute neurovisceral attacks and chronic symptoms. In ENVISION (NCT03338816), givosiran treatment for 6 months reduced attacks and other disease manifestations compared with placebo. Herein, we report data from the 36-month final analysis of ENVISION. METHODS: Ninety-four patients with AHP (age ≥12 years) and recurrent attacks were randomized 1:1 to monthly double-blind subcutaneous givosiran 2.5 mg/kg (n = 48) or placebo (n = 46) for 6 months. In the open-label extension (OLE) period, 93 patients received givosiran 2.5 or 1.25 mg/kg for 6 months or more before transitioning to 2.5 mg/kg. Endpoints were exploratory unless otherwise noted. RESULTS: During givosiran treatment, the median annualized attack rate (AAR) was 0.4. Through Month 36, annualized days of hemin use remained low in the continuous givosiran group (median, 0.0 to 0.4) and decreased in the placebo crossover group (16.2 to 0.4). At end of OLE, in the continuous givosiran and placebo crossover groups, 86% and 92%, respectively, had 0 attacks. AAR was lower than historical AAR in 98% and 100%, respectively (post hoc analysis), and there were 0 days of hemin use in 88% and 90%, respectively. The 12-item short-form health survey physical and mental component summary scores increased by 8.6 and 8.1, respectively (continuous givosiran) and 9.4 and 3.2, respectively (placebo crossover). EQ-5D health-related questionnaire scores increased by 18.9 (continuous givosiran) and 9.9 (placebo crossover). Lower urinary delta-aminolevulinic acid and porphobilinogen levels were sustained. Safety findings demonstrated a continued positive risk/benefit profile for givosiran. CONCLUSIONS: Long-term monthly givosiran treatment provides sustained and continued improvement in clinical manifestations of AHP. GOV IDENTIFIER: NCT03338816. EUDRACT NUMBER: 2017-002432-17. IMPACT AND IMPLICATIONS: Acute hepatic porphyria (AHP) is a group of rare, chronic, multisystem disorders associated with overproduction and accumulation of neurotoxic heme intermediates (delta-aminolevulinic acid and porphobilinogen), sometimes resulting in recurrent acute attacks and long-term complications. Givosiran, a small-interfering RNA that prevents accumulation of delta-aminolevulinic acid and porphobilinogen, is approved for the treatment of AHP. These final 36-month results of ENVISION, a phase III study of givosiran in patients with AHP and recurrent attacks, show that long-term monthly treatment with givosiran leads to continuous and sustained reductions in annualized attack rate and use of hemin over time, as well as improved quality of life, with an acceptable safety profile. These results are important for physicians, patients, families, and caregivers who are grappling with this debilitating and potentially life-threatening disease with few effective and tolerable treatment options.

Solar urticaria - An Australian case series of 83 patients.

Solar urticaria (SU) is a rare form of urticaria with a pathogenesis that is poorly understood. It affects all skin types, can be difficult to diagnose, and is challenging to manage effectively. We conducted a retrospective review of patients with SU in our institution. A total of 83 patients (56 females) were identified as having SU. The mean age was 32 years (7-74) at first development of symptoms/signs of SU. Pruritus was the most common symptom reported (79%). Of the 60 patients who underwent monochromator testing at least once, 35 had SU confirmed with most reacting to visible light and UVA, or to UVA alone. Antihistamines and sun avoidance remain the mainstay treatment for SU but other treatments, including omalizumab, are of potential interest in treating patients with recalcitrant SU. The characterisation of this large case series of patients may help dermatologists recognise and manage this rare disorder appropriately.

Haem arginate as effective maintenance therapy for hereditary coproporphyria.

A 35-year-old woman presented with skin fragility and photosensitivity with blisters affecting her face and hands. Other symptoms included intermittent headache, fatigue, abdominal pain and nausea. Porphyrin studies were markedly raised, with features consistent with hereditary coproporphyria (HCP). Despite strict precautions, symptoms remained significantly problematic. Regular haem arginate infusions of 3 mg/kg per day over 4 days on a monthly basis were commenced and resulted in significant improvement of the patient's symptoms and a reduction in urinary porphobilinogen. Although haem arginate infusion is known as a treatment for severe acute attacks of HCP, the effectiveness of regular infusions as maintenance therapy has not been established. This is the first report of effective symptom control correlating with normalization of biochemical markers in a patient receiving regular haem arginate infusions for the treatment of HCP.

Photodynamic therapy for Basal cell carcinoma in recessive dystrophic epidermolysis bullosa.

A 22-year-old male with recessive dystrophic epidermolysis bullosa with a large superficial and nodular basal cell carcinoma on his right forehead was treated with photodynamic therapy. The treatment was well tolerated, and the site healed well. Patients with epidermolysis bullosa are at increased risk of developing skin cancers, particularly squamous cell carcinomas. However, basal cell carcinomas are rare in recessive dystrophic epidermolysis bullosa. As patients with epidermolysis bullosa have recurrent blistering and poor wound healing, surgery may not be the optimal choice in treating skin cancers. We present this case to highlight that photodynamic therapy may be a helpful and safe technique in the treatment of superficial skin cancers in patients with epidermolysis bullosa, as an alternative to more radical methods.

Actinic prurigo.

Actinic Prurigo (AP), an uncommon idiopathic photodermatosis, presents a distinct clinical picture and can be severely debilitating. The clinical features, investigation and treatment of AP are reviewed. We report the experience of an Australian photobiology unit with this condition.

A case of PHACE syndrome.

A young girl with PHACE syndrome presented with a posterior fossa malformation, a segmental telangiectatic right facial haemangioma, a novel aortic arch anomaly, a congenital right fourth cranial nerve palsy (not previously described in this syndrome) and Horner's syndrome. Hydrocephalus was limited to the intrauterine period and detection of the cardiovascular anomalies was a direct result of recognition of this syndrome. She has received laser treatment for the haemangioma and is awaiting surgical correction of her cranial nerve palsy.

Epidemiology and impact of childhood molluscum contagiosum: a case series and critical review of the literature.

Parents of 30 children with clinically diagnosed molluscum contagiosum were surveyed to assess their perception of the condition, its treatment, its impact on their everyday lives, and on the children themselves. Among parents, 82% reported that molluscum contagiosum concerned them moderately or greatly. Concerns focused on physical issues associated with the infection, such as scarring, itching, chance of spread to peers, pain, and the effects of treatments. Quality of life was not affected. Molluscum contagiosum was most common among school-age children. Eighteen of 29 respondents swam in public pools, a common activity in children of this age. All epidemiologic studies of molluscum contagiosum in otherwise healthy individuals, published since 1966, have been critically reviewed herein. The review confirms an association between swimming pool use and molluscum contagiosum. Age, living in close proximity, skin-to-skin contact, sharing of fomites, and residence in tropical climates were also associated with higher rates of infection while sex, seasonality, and hygiene showed no such association.

Eczema--practical management issues.

BACKGROUND: Eczema is a common, and at times challenging, condition to manage. It often involves an irritable child, concerned parents and numerous return visits. A variety of mainstream and alternative treatments can confuse both the clinician and patient. OBJECTIVE: This article aims to refresh readers by reviewing evidenced based treatment protocols, and exploring some of the new and evolving treatments in eczema. DISCUSSION: Managing eczema is a multifocal task involving a variety of medicinal and practical approaches. Education of parents and carers is critical. Having a clear plan that can be adapted to each patient's needs is helpful for all involved, with management focussing on control rather than cure. Simple emollients and preventive measures are used to reduce heat, dryness and prickling of the skin. Topical corticosteroids remain the gold standard in the treatment of the inflammation of eczema. However, combining topical steroids with the new calcineurin inhibitors, wet dressings and behavioural modification should increase the time interval between exacerbations.

Combination topical treatment of molluscum contagiosum with cantharidin and imiquimod 5% in children: a case series of 16 patients.

The objective of this study was to assess the efficacy and tolerability of combination therapy for molluscum contagiosum (MC) with topical cantharidin and imiquimod 5%. A prospective case series of 16 paediatric patients with a mean age of 4.8 years had cantharidin applied to lesions by a dermatologist, followed by home treatment with imiquimod 5% cream nightly for an average of 5 weeks. This regimen resulted in >90% of lesions clearing in 12 patients, with half of these being totally clear. Two patients had 80-90% of lesions resolve. Two patients had 30-50% clearance of lesions at the end of the treatment period. One patient found the cantharidin reaction too strong. The mean number of imiquimod 250 mg sachets used was 4.25. In conclusion, this study suggests that combination therapy using cantharidin and imiquimod for treatment of MC in children is effective and well tolerated.