[CVID is a multifaceted disease].

Common variable immunodeficiency disease (CVID) is the most common primary immunodeficiency in adults, and multiple organs may be involved. This is a case report of a 49-year-old female patient with granulomatous-lymphocytic interstitial lung disease, liver fibrosis, portal hypertension and rectal cancer. Examinations showed non-necrotic granulomas in her lungs, mediastinal glands and liver, and she was seen in six different specialities. The multifaceted manifestation of CVID calls for multidisciplinary collaboration.

Birt-Hogg-Dubé syndrome: spontaneous pneumothorax as a first symptom.

Birt-Hogg-Dubé syndrome (BHDS) is a rare hereditary autosomal dominant condition characterised by benign cutaneous lesions, lung cysts, increased risk of spontaneous pneumothorax and renal cancer. It shows great heterogenous presentation within and between affected families. We report a case of a Danish female patient with recurrent pneumothoraces as the first symptom of BHDS. Over the years, she developed skin changes, and a family history of skin changes, pneumothoraces and renal cancer was discovered. BHDS was suspected, a genetic analysis was performed and a pathogenic variation c.1285delC in FLCN gene was detected in the patient. As we stated the diagnosis BHDS, we discovered several undiagnosed family members all of them now entering a lifelong follow-up programme with abdominal imaging because of the increased risk of developing renal cancer. BHDS should be known to oncologists, dermatologists and pulmonologists as the patients most often present to these medical disciplines.

Optimised diagnosis and treatment of necrotizing external otitis is warranted.

INTRODUCTION: Necrotizing external otitis (NEO) is a devastating and life-threatening infection in the external auditory canal and the temporal bone. The aim of this paper is to evaluate the diagnostics and treatment of NEO and to recommend new guidelines. MATERIAL AND METHODS: Eleven patients were retrospectively enrolled under the code DH609 otitis externa without specification from 1 January 2000 to 31 December 2009. Records were reviewed to register: age, symptoms, clinical findings, comorbidity, imaging, microbiology and treatment. RESULTS: The median age was 75 years and the median time of therapy at the hospital was 6.3 months. All patients belonged to a risk group. A diagnostic delay was found resulting in further progression of the disease. In contrast to current international recommendations, the treatment consisted mostly of local antibiotics in combination with surgery. All patients survived, but most patients were left with hearing loss and psychiatric problems. CONCLUSION: A greater awareness of diagnostic criteria and a shift from local antibiotics to prolonged systemic monotherapy with ciprofloxacin in accordance with international concepts is recommended. Surgery should be left for extensive and refractory cases only. A list of diagnostic criteria and treatment guidelines is presented. FUNDING: Not relevant. TRIAL REGISTRATION: Not relevant.