Objective. To asses a cohort of 105 consecutive patients with angiotensin converting enzyme-inhibitor induced angioedema with regard to demographics, risk factors, family history of angioedema, hospitalization, airway management, outcome, and use of diagnostic codes used for the condition. Study Design. Cohort study. Methods. This was a retrospective cohort study of 105 patients with angiotensin converting enzyme-inhibitor induced angioedema in the period 1995–2014. Results. The cohort consisted of 67 females and 38 males (F : M ratio 1.8), with a mean age of 63 [range 26–86] years. Female gender was associated with a significantly higher risk of angiotensin converting enzyme-inhibitor induced angioedema. 6.7% had a positive family history of angioedema. Diabetes seemed to be a protective factor with regard to angioedema. 95% experienced angioedema of the head and neck. 4.7% needed intubation or tracheostomy. 74 admissions took place during the study period with a total of 143 days spent in the hospital. The diagnosis codes most often used for this condition were “DT783 Quincke's oedema” and “DT78.4 Allergy unspecified”. Complement C1 inhibitor was normal in all tested patients. Conclusion. Female gender predisposes to angiotensin converting enzyme-inhibitor induced angioedema, whereas diabetes seems to be a protective factor.

Background Supportive care is the cornerstone of management of adult and paediatric Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). However, consensus on the modalities of supportive care is lacking. Objectives Our aim in this international multicentric Delphi exercise was to establish a multidisciplinary expert consensus to standardize recommendations regarding supportive care in the acute phase of SJS/TEN. Methods Participants were sent a survey via the online tool SurveyMonkey, consisting of 103 statements organized into 11 topics: multidisciplinary team composition, suspect drug management, infection prevention, fluid resuscitation and prevention of hypothermia, nutritional support, pain and psychological distress management, management of acute respiratory failure, local skincare, ophthalmological management, management of other mucosa, and additional measures. Participants evaluated the level of appropriateness of each statement on a scale of 1 (extremely inappropriate) to 9 (extremely appropriate). The results were analysed according to the RAND/UCLA Appropriateness Method. Results Forty-five participants from 13 countries (on three continents) participated. After the first round, a consensus was obtained for 82.5% of the 103 initially proposed statements. After the second round, a final consensus was obtained for 102 statements. Conclusions We have reached an international Delphi-based consensus on best supportive care practice for SJS/TEN. Our expert consensus should help guide physicians in treating patients with SJS/TEN and thereby improve short-term prognosis and the risk of sequelae.What is already known about this topic?• Supportive care is the cornerstone of management of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) in the acute phase.• There is no consensus or guideline on the best supportive care.

Hereditary leiomyomatosis and renal cell cancer (HLRCC) is an inherited tumour predisposition syndrome. Around 75% of individuals with HLRCC develop cutaneous leiomyomas, which are skin tumours that can cause pain and itching. Most women with HLRCC develop uterine leiomyomas that often cause gynecological symptoms and typically require surgery. Around 20% develop renal cell carcinoma. HLRCCassociated renal cell carcinomas are aggressive tumours with a high potential to metastasize. Individuals with features suggestive of HLRCC and at-risk family members should undergo genetic testing. Individuals harbouring a pathogenic mutation should be offered lifelong surveillance so renal neoplasms are detected and treated in time. Hereditary leiomyomatosis and renal cell cancer is a genodermatosis with an autosomal dominant inheritance pattern. It is a tumour predisposition syndrome characterized by cutaneous and uterine leiomyomas, and increased susceptibility to develop renal cell carcinoma. There are 200-300 families with hereditary leiomyomatosis and renal cell carcinoma reported worldwide, but the syndrome is believed to be underdiagnosed. Cutaneous leiomyomas are small smooth muscle tumours that tend to grow over time. Larger lesions, in particular, can cause pain or itching. Uterine leiomyomas have a high penetrance in women with hereditary leiomyomatosis and renal cell cancer. They frequently cause symptoms, and surgical intervention is often necessary. Hereditary leiomyomatosis and renal cell cancer-associated renal cell carcinomas have a high potential to metastasize. Patients are diag nosed by genetic testing if a pathogenic mutation is demonstrated in the gene encoding fumarate hydratase. Immunohistochemistry may be a useful diagnostic approach in patients without a detectable pathogenic mutation. Diagnosed patients should be monitored for renal tumours in a lifelong surveillance programme.

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We report a case of acute dystonia of the face, jaw and tongue caused by metoclopramide and mimicking angioedema. The patient had attacks for several years before the correct diagnosis was made and we present the first ever published video footage of an attack. This adverse drug reaction is known, but might be underdiagnosed since it can mimic a wide range of other diseases.

Treatment with Tenutex ® should be considered in patients diagnosed with scabies if the first-line treatment with 5% permethrin fails. Treatment failure with 5% permethrin may be due to tolerance or resistance in scabies mites.

Hypereosinophilic syndrome (HES) was first described in 1968 by Hardy and Anderson. 1 According to the World Health Organization (WHO), IHES is characterized by eosinophilia greater than 1.5 × 10 9 /L for at least 6 months and the presence of end-organ damage. In children, hypereosinophilia often occurs secondary to parasitic infections, medications, atopic dermatitis, and primary immunodeficiency like hyper-IgE syndrome, and rarely occurs due to graft-versus-host disease and neoplastic disease. 2 The prevalence and incidence of IHES are unknown, but it is considered a rare disease in children and mainly affected adults between 20 and 50 years old with a 9:1 male-female ratio. 3,4 Hypereosinophilia can lead to cell damage and organ dysfunction due to the release of granule proteins. 5 Here, we report a pediatric case of IHES with severe eosinophilia, intense pruritus, and a generalized papulonodular skin rash. 2 | CASE REPORT A 10-year-old boy with parents of Kurdish origin presented with a generalized, itchy, papulonodular skin rash at the outpatient clinic of dermatology and pediatrics at Aarhus University Hospital. The patient had allergic rhinitis with type 1 allergies to birch, cats, peanuts, and dust mites. There was no family history of atopy or eosinophilia. The parents were nonconsanguineous, and the two older siblings were healthy. At birth, a pustular rash was observed on the face and back, which was diagnosed as erythema toxicum neonatorum.