Preface

We are increasingly exposed to chemicals, toxins, and nuclear and electronic radiation, either through our food or our environment. Environmental and climatic factors can cause disease outcomes, and rates of occupational and technology-induced pathologies have surpassed those of communicable diseases. Globally, people in different work environments and climates are experiencing unexpected health issues not previously envisaged. Office workers who sit for long hours working with computers or other electronic devices report low back and spine pain as well as issues with eyesight. Workers exposed to asbestos and cement dust have been diagnosed with lung diseases and inhalation problems, including some forms of cancer. Those exposed to localized environments of extreme heat may develop heat rash, heat edema, heat syncope, cramps, exhaustion, life-threatening heatstroke, and other related ailments. Some ailments may not yet have a known origin and these diseases require more research to ascertain their causes and treatments. This book provides insight into rare diseases of public health importance.

Chapter 1, "Introductory Chapter: Possible Occupational, Technological and Climatic Contributions to Rare Diseases Occurance", reviews the effects of technology, climate, and occupation on health. For example, contact with environmental toxins such as soot, nitrates, NH3 and nitrogenous gaseous oxides (NOx), sulphur oxides, aerosols, CH3, and particulate matter activate biomarkers that cause enzyme hormonal distortion, inhibition of fetal growth, and DNA damage. The chapter focuses on technologyinduced and occupational hazards such as exposure to chemicals, machines, noise, and radiation, as well as climatic causes of rare diseases.

Chapter 2, "A Short Overview of Behcet's Disease", describes Bechet's as a chronic, relapsing-remitting, occlusive vasculitis affecting multiple organ systems characterized by oral and genital aphthous ulcers, arthritis, cutaneous lesions, and ocular, gastrointestinal, and neurological manifestations. Behcet's disease etiology remains uncertain, but various studies suggest an infectious trigger with inflammatory mediators and immune deregulation as the cause of a genetically susceptible host. Environmental factors such as exposure to organophosphates, heavy metals, organochlorides, and allergens exacerbate Behcet's disease.

Chapter 3, "Entero-Behcet: A Challenging Aspect of Behçet's Disease", discusses entero-Behcet's, a primitive vasculitis of unknown origin featuring recurrent oral and/ or genital aphthosis, ocular manifestations, and arthritis. Intestinal presentation of the disease is rare; prevalence is about 5%–10%. It may be very severe and is difficult to diagnose and differentiate from inflammatory bowel disease, especially when it is the initial manifestation. Genetic factors, which cannot completely explain the emergence of Behcet's disease, include the human leukocyte antigen (HLA)-B51 allele, ERAP 1, and MHC class I-related gene A (MICA).

Chapter 4, "Genetics of Behçet's Disease," discusses the genetics of Behçet's disease. Although pathogenesis remains uncertain, genome-wide and validation studies have shown that genetic predisposition is a major factor in disease susceptibility. Behçet's disease is diagnosed worldwide, but the highest prevalence is reported in countries stretching from Japan to the Mediterranean region along the ancient trading route the Silk Road.

Chapter 5, "Malignant Mesothelioma *In Situ*: A Controversial Diagnostic Entity – A Review", examines the aggressiveness of malignant serosal surfaces strongly associated with asbestos exposure. Malignant mesothelioma can be difficult to distinguish from reactive benign hyperplasia. Mutations in BAP-1 and CDKN2a distinguish mesothelioma from reactive hyperplasia. Malignant mesothelioma is a rare malignancy of serosal-lined surfaces and the tumors can potentially invade local tissues and metastasize to distant sites. The World Health Organization's (WHO) newest classification of thoracic malignancies adds malignant mesothelioma in situ as a distinct diagnostic entity.

Chapter 6, "Diagnosis of Dentofacial Anomalies", examines dentofacial anomalies, including clinical features, diagnostic criteria, and investigation protocols. These anomalies involve the dentofacial region and may be related to teeth, maxilla, mandible, and soft tissue. They are syndromic conditions that can spread to various other vital organs. Alterations of craniofacial form, structure, or function are the cardinal features of these conditions. Therefore, clinicians should be knowledgeable about dysmorphological changes in orofacial structures so they can consider certain specific disease entities and rule out the involvement of other tissues and organ systems that may present similarly.

Chapter 7, "Neuropathic Pruritus: An Underrecognized and Often Misdiagnosed and Difficult to Treat Medical Condition", reviews case reports, clinical trials, cohort studies, systematic reviews, and meta-analyses associated with neuropathic pruritus. Statistical estimates suggest that about 8% of chronic pruritic cases are of neuropathic origin. Damage to any part of the peripheral and central somatosensory system can lead to neuropathic pruritus. Common neuropathic pruritic syndromes include different clinical presentations such as postherpetic pruritus, trigeminal trophic syndrome, anogenital pruritus, scalp dysesthesia, nerve compression syndromes (e.g., notalgia paresthetica, brachioradial pruritus), and small-fiber neuropathy (secondary to various metabolic, infectious, autoimmune, and genetic diseases).

Chapter 8, "Lipoatrophia Semicircularis", discusses a rare, benign, and reversible subcutaneous tissue atrophy that mostly affects women. It appears mainly on the anterior and lateral regions of the thighs and consists of unilateral, bilateral transverse, semi-circular, and depressed bands appearing approximately 72 cm from the ground. The cause is not yet known, but an unproven hypothesis suggests circulatory abnormalities, microtraumas, wearing of tight trousers, exposure to electromagnetic fields in the work environment, or electrostatic charges generated from computers or printers as possible causes of bioelectric changes in the skin.

Chapter 9, "Short QT Syndrome: Update on Genetic Basis", describes short QT syndrome (SQTS) as an extremely rare inherited arrhythmogenic entity; it affects less than 200 families worldwide. The prevalence estimate is less than 1/10.000 in adults and about 1/2000 in children and adolescents. SQTS is potentially lethal for children in the first year of life. It leads to cardiac arrest in close to 4% of patients, making it

**V**

one of the causes of sudden infant death syndrome (SIDS). SQTS is characterized by a short QT interval when ECG shows asymmetric and sharp T waves in the precordial area. Resting ECG should be performed at a normal heart rate when SQTS is suspected Ventricular and atrial fibrillation are present in most patients and cardiac events usually occur in adrenergic situations (noise or exercise). The most common symptoms are palpitations (30%), syncope (25%), and cardiac arrest (40%).

The editors wish to thank the authors for their hard work, faith in us, and worthy contributions. We are also thankful for the staff at IntechOpen, especially Author Service Manager Ms. Nika Karamatic. Finally, we thank our families for their under-

Editor Nduka is grateful to his wife Ogochukwu and his children Chimerem-mma,

**John Kanayochukwu Nduka**

Nnamdi Azikiwe University,

Department of Dermatology, Dokuz Eylül University,

Awka, Nigeria

**Sevgi Akarsu** Faculty of Medicine,

Izmir, Turkey

Environmental Chemistry and Toxicology Research Unit,

Pure and Industrial Chemistry Department,

standing and support throughout the process of editing this book.

Onyinye, and Chisom.

one of the causes of sudden infant death syndrome (SIDS). SQTS is characterized by a short QT interval when ECG shows asymmetric and sharp T waves in the precordial area. Resting ECG should be performed at a normal heart rate when SQTS is suspected Ventricular and atrial fibrillation are present in most patients and cardiac events usually occur in adrenergic situations (noise or exercise). The most common symptoms are palpitations (30%), syncope (25%), and cardiac arrest (40%).

The editors wish to thank the authors for their hard work, faith in us, and worthy contributions. We are also thankful for the staff at IntechOpen, especially Author Service Manager Ms. Nika Karamatic. Finally, we thank our families for their understanding and support throughout the process of editing this book.

Editor Nduka is grateful to his wife Ogochukwu and his children Chimerem-mma, Onyinye, and Chisom.
