Dermoscopic pattern of Kyrle's disease.

The clinical diagnosis of Kyrle's disease may sometimes be challenging, due to the clinical similarity of lesions to other pruritic dermatosis. Although the dermoscopy is being increasingly used in daily practice, there is insufficient data in literature describing the dermoscopic patterns of Kyrle's disease, since only one report has been published to date. Herein we report our dermoscopic observation with additional diagnostic tips in a case who was diagnosed with Kyrle's disease histopathologically.

Darier disease is associated with heart failure: a cross-sectional case-control and population based study.

Human data supporting a role for endoplasmic reticulum (ER) stress and calcium dyshomeostasis in heart disease is scarce. Darier disease (DD) is a hereditary skin disease caused by mutations in the ATP2A2 gene encoding the sarcoendoplasmic-reticulum Ca2+ ATPase isoform 2 (SERCA2), which causes calcium dyshomeostasis and ER stress. We hypothesized that DD patients would have an increased risk for common heart disease. We performed a cross-sectional case-control clinical study on 25 DD patients and 25 matched controls; and a population-based cohort study on 935 subjects with DD and matched comparison subjects. Main outcomes and measures were N-terminal pro-brain natriuretic peptide, ECG and heart diagnosis (myocardial infarction, heart failure and arrythmia). DD subjects showed normal clinical heart phenotype including heart failure markers and ECG. The risk for heart failure was 1.59 (1,16-2,19) times elevated in DD subjects, while no major differences were found in myocardial infarcation or arrhythmias. Risk for heart failure when corrected for cardivascular risk factors or alcohol misuse was 1.53 (1.11-2.11) and 1.58 (1,15-2,18) respectively. Notably, heart failure occurred several years earlier in DD patients as compared to controls. We conclude that DD patients show a disease specific increased risk of heart failure which should be taken into account in patient management. The observation also strenghtens the clinical evidence on the important role of SERCA2 in heart failure pathophysiology.

Dermatoscopy and Reflectance Confocal Microscopy Correlations in Nonmelanocytic Disorders.

Dermatoscopy and in vivo reflectance confocal microscopy are noninvasive techniques that provide a horizontal approach, with an en face view of the skin structures. Both techniques assist in the clinical diagnosis of a variety of inflammatory and infectious cutaneous disorders. In many cases, they have shown concordance. Their combined use represents, in several instances, a promising option to reach the final diagnosis without the need for invasive procedures.

Dermoscopic pattern of Kyrles disease

Abstract The clinical diagnosis of Kyrle's disease may sometimes be challenging, due to the clinical similarity of lesions to other pruritic dermatosis. Although the dermoscopy is being increasingly used in daily practice, there is insufficient data in literature describing the dermoscopic patterns of Kyrle's disease, since only one report has been published to date. Herein we report our dermoscopic observation with additional diagnostic tips in a case who was diagnosed with Kyrle's disease histopathologically.

[Esophageal localizations of Darier's disease. Radiological description apropos of 4 cases]. / Localizaciones esofágicas de la enfermedad de Darier. Descripción radiológica. A propósito de cuatro casos.

The oesophagic affectation seems very frequent in Darier's disease. The authors have found oesophagic lesions in 4 cases examined. The radiologic aspect of this localization shows an oesophagic border in "post stamp" and a definite irregularity of the fasciculation. This fibroscopic examination shows the presence of keratotic papules similar to the skin lesions of this disease.

[An inverse course of Darier dyskeratosis follicularis]. / Eine inverse Verlaufsform der Dyskeratosis follicularis Darier.

A 56-year-old female patient had suffered from isolated, unclassified palmoplantar keratosis since childhood. During the climacteric horny papules appeared for the first time on the extremities and trunk. Histologically, the efflorescences on the body and the palmoplantar keratosis were classifiable as keratosis follicularis. Predilection sites of Darier's disease were spared. Altogether, the usual pattern of involvement was reversed.

Symptomatic and asymptomatic salivary duct abnormalities in Darier's disease: a sialographic study.

Symptoms of obstructive sialadentis have been occasionally reported as a feature of Darier's disease. Parotid sialograms of three patients with confirmed Darier's disease and histories suggestive of salivary obstruction revealed dilatation with periodic strictures and indentations affecting the main ducts. Similar parotid sialograms in three Darier's disease patients with no symptoms attributable to their salivary glands revealed seepage of contrast media associated with the secondary and tertiary ducts. These findings suggest that salivary glands may be more commonly involved in Darier's disease than previous reports indicate.