An Update on the Cutaneous Manifestations of Darier Disease.

BACKGROUND: Knowledge about the clinical features of Darier disease, an orphan autosomal-dominant genetic disorder, is sparse and has been evaluated only in few studies. OBJECTIVES: To investigate the clinical features of a large group of patients with Darier disease, and to explore for associations between disease characteristics and severity of the disease. METHODS: Seventy-six individuals with Darier disease were evaluated utilizing a structured questionnaire-based interview, a physical examination, and a retrospective assessment of their medical records. RESULTS: The most frequent locations of lesions were hands (99%) and fingernails (93%). Wart-like lesions on the hands were more visible after soaking them in water for 5 minutes, we therefore named this phenomenon the "wet hand sign". Oral involvement was found in 43% of patients, while 48% of women and 16% of men showed genital lesions. Patients with severe Darier disease had a tenfold greater risk of developing genital lesions than those with mild disease (P = .01). Most patients (88%) in our study exhibited a combination of the four types of the disease patterns of distribution (flexural, seborrheic, nevoid, and acral). CONCLUSIONS: Documentation of disease on the hands and fingernails provides a highly sensitive means to aid in the diagnosis of Darier disease. It is important to evaluate mucosal lesions including genital and oral mucosa.

Hospital Operation During a Disaster - Hospital Multi-Component Emergency Center (HMCEC).

A hospital's operating modes can be divided into three main levels of operation: routine, mass-casualty, and interim states that require procedures and resources beyond the routine capacity.Regardless of the nature of the event and the needs, the medical staff has to be prepared for a sudden increase in demand for service that could overwhelm the functional capacity and safety standards of the hospital.In any sort of an emergency event, the hospital has to fulfill two goals: First is to be able to sustain itself against the sudden rise in demand for medical support; the aid given depends on the nature of the disaster. The second goal is to continue supporting the essential routine duties for patients already hospitalized and for others arriving at the hospital regardless of the disaster.The aim of this paper is to describe the principles and the methods for hospital operation in case of a disaster-level event. The paper describes the structure and methods for handling prolonged disaster-level events and the adjustments that can be made in the case of lower intensity events.