Localized Darier disease: three cases of Type 1 segmental mosaicism.

Darier disease (DD) is an autosomal dominant acantholytic dermatosis with an estimated prevalence of 1 in 30 000-100 000. A localized form of DD was first described by Kreibich in 1906 and is thought to account for 10% of all cases. A number of clinical variants have been reported including: unilateral, linear, segmental or zosteriform DD.

Programmed death ligand 1 expression in EBUS aspirates of non-small cell lung cancer: Is interpretation affected by type of fixation?

Background: Much of the reluctance about using cytology specimens rather than histology specimens to assess programmed death ligand 1 (PD-L1) expression for guiding the use of immune modulating drugs in the management of non-small cell lung cancer (NSCLC) is based on the belief that the alcohol-based fixatives favored by cytopathologists might reduce the antigenicity of PD-L1 and lead to artifactually low expression levels and false-negative reporting. Therefore, this study was performed to determine whether there is any difference in PD-L1 expression between endobronchial ultrasound (EBUS)-guided aspirates of NSCLC fixed in alcohol-based fixatives and those fixed in neutral buffered formalin (NBF), the standard laboratory fixative for histology specimens.

Methods: The expression of PD-L1 was compared in 50 paired EBUS aspirates of NSCLC taken from the same lymph node during the same procedure.

Elevated CA 19-9 levels observed in association with a pulmonary neuroendocrine tumour and amyloid.

Investigations completed in a 77-year-old ex-smoker presenting with weight loss showed raised CA 19-9 levels. The findings of chest radiograph, abdominal computed tomography (CT) and gastrointestinal endoscopic examinations were all normal. On follow-up, the patient developed left upper lobe collapse on chest radiograph with increasing CA 19-9 levels.

Leiomyosarcoma of the tonsil.

Objective: We report a case of leiomyosarcoma arising in the tonsil in a 73-year-old man.

Method: Case report and review of the English language literature (using Pubmed, Ovid and Proquest databases).

Results: To our knowledge, this is the first case of tonsillar leiomyosarcoma to be reported in the English language literature.

An unusual case of low-grade tubulopapillary adenocarcinoma of the sinonasal tract.

Background: Low-grade papillary adenocarcinomas of the sinonasal tract are rare neoplasms. Over recent years, little doubt remains that this tumour represents a separate entity based on morphology, ultrastructural features and behaviour. We outline a case of this rare entity displaying a not hitherto described immunophenotype.

Successful treatment of type I adult-onset pityriasis rubra pilaris with infliximab.

A 59-year-old woman presented with a painful, pruritic eruption that had commenced as an erythematous, dry patch on the upper back but progressed to erythroderma. Examination revealed orange-tinged erythroderma, scalp scaling, ectropion, palmoplantar keratoderma and nail changes. A diagnosis of type I adult-onset pityriasis rubra pilaris was made, and a subsequent skin biopsy was consistent with this.