bluuebunny/crossref_metadata_2025_split huggingface dataset:
Poikiloderma Syndromes
Authors: Irvine Alan D. , Mellerio Jemima E. 
Published: August 2023

Abstract
Characterized by mixed hypo‐ and hyperpigmentation, skin atrophy and telangiectasia, poikiloderma is a feature of a number of different genetic skin diseases including dyskeratosis congenita, Rothmund–Thomson syndrome, Kindler syndrome and poikiloderma with neutropenia. Systemic associations of these disorders may include developmental abnormalities, a predisopsition to malignancy or infection, or progressive fibrosis of the bone marrow and other organs. Clinical and, where possible, genetic evaluation are vital to anticipation of potential complications, targeted management and genetic counselling.
Hamartoneoplastic Syndromes
Authors: Irvine Alan D. , Mellerio Jemima E. 
Published: August 2023

AbstractThis chapter covers the four most common hamartoneoplastic syndromes, including neurofibromatosis type 1, tuberous sclerosis, Gardner syndrome and Cowden syndrome. These conditions have diverse clinical and genetic features and require detailed condition‐specific knowledge, along with specific treatment guidelines and plans. They are, however, united by the common features of extensive and diverse skin manifestations along with somatic hamartomas and a significant risk of cutaneous and non‐cutaneous neoplasia. The four conditions are reviewed in detail with respect to pathophysiology, clinical features, diagnosis and management. A brief review of the emerging field of disorders with the collective term ‘RASopathies’ is included.
Acquired Disorders of Hair
Authors: Messenger Andrew G. , Sinclair Rodney D. , Farrant Paul , de Berker David A. R. 
Published: August 2023

AbstractHair is a defining feature of mammals. In other species hair confers important functions that affect survival. Most have been lost or are irrelevant in humans but the role of hair in social and sexual signalling in women and in men survives and thrives. Departures from cultural norms, either physiological or due to pathology, can therefore cause much concern and anxiety. Following an introduction to hair biology, this chapter considers the approach to the diagnosis and management of the patient with hair loss before discussing specific hair disorders. These include the various forms of hair loss due to hair follicle pathology, disturbances in hair cycling and hair shaft dystrophies, and disorders associated with excessive hair growth. The chapter concludes with a discussion of acquired alterations in hair pigmentation.
Hidradenitis Suppurativa
Authors: Desai Nemesha , van der Zee Hessel H. , Jemec Gregor B. E. 
Published: August 2023

AbstractHidradenitis suppurativa is characterized by inflamed lesions and scars that are restricted to typical topography (axillae, inframammary folds and groin), with a chronic course with flares. The average age of onset is after puberty and it is estimated to affect 1–2% of the European population. The aetiology is not known, but hereditary factors, obesity, smoking, host defence mechanisms, sex hormones and bacterial colonization are each suspected to play a role. Histology shows it to be a disease of the hair follicle. Flares are characterized by pain and suppuration, and the disease has a strong negative impact on patients' quality of life. Basic treatment should include supportive measures (weight loss, bandages and analgesics) for all patients. It is recommended that patients are treated with a combination of medical and surgical therapies. Medical therapies include antibiotics and anti‐inflammatory drugs. Surgical therapies include non‐ablative and ablative lasers as well as excisions.
Acquired Disorders of the Nails and Nail Unit
Authors: de Berker David A. R. , Richert Bertrand , Baran Robert 
Published: August 2023

AbstractThis chapter deals with all aspects of the nails in health and disease apart from hereditary nail disorders and fungal infections of the nails which are addressed in Chapters 69 and 32, respectively. Nail biology and growth establishes the basis for an understanding of how the nail may be affected by disease processes and their treatment. The significance of nail signs, which may be an important indicator of systemic diseases, is fully discussed before individual disorders of the nail and perionychium are described. The section on dermatoses affecting the nails includes the main categories of inflammatory and neoplastic diseases seen at other sites in the skin, but as expressed in the nail unit. The diagnostic imaging of the nail and nail surgery sections highlight special considerations required in managing disorders of the nail. Finally, the reader is introduced to cosmetic procedures used on nails and problems which may arise from them.
Acquired Disorders of Dermal Connective Tissue
Authors: Lovell Christopher R. 
Published: August 2023

AbstractThe subject matter of this chapter comprises the major acquired disorders that affect mainly collagen and elastic tissue. The connective tissue of normal skin is discussed elsewhere. Heritable disorders of connective tissue, such as Ehlers–Danlos syndrome, are covered in another chapter, as are disorders of ground substance, such as the mucinoses and mucopolysaccharidoses. In addition, many disorders, including developmental defects, neoplasms and other reactive or scarring conditions, may involve the connective tissues. Although it can be convenient to separate conditions into disorders affecting collagen or elastic tissue, the distinction is somewhat artificial, as changes in these proteins are generally preceded by abnormal metabolism of other matrix components, such as fibronectin or periostin.
Granulomatous Disorders of the Skin
Authors: Bourke Johnny 
Published: August 2023

AbstractGranulomatous inflammation is seen in a wide range of skin conditions including infections, neoplasms and inflammatory disorders. This chapter addresses three important idiopathic granulomatous disorders of the skin. The first two, granuloma annulare and necrobiosis lipoidica, are relatively common. Treatment is of uncertain value and is generally disappointing. Granuloma annulare usually resolves spontaneously over years but necrobiosis lipoidica tends to persist. Their association with systemic disorders is discussed. The third, cutaneous Crohn disease, is uncommon. It may affect the skin by direct extension causing swelling and ulceration or occasionally may involve distant sites, so‐called metastatic Crohn disease. Cutaneous sarcoidosis is addressed in another chapter.
Sarcoidosis
Authors: Marcoval Joaquim , Mañá Juan 
Published: August 2023

AbstractSarcoidosis is an antigen‐mediated disease of unknown aetiology characterized by the presence of non‐caseating epithelioid cell granulomas in multiple organs. It involves mainly the lungs, mediastinal and peripheral lymph nodes, eyes and skin. Less frequent but usually severe manifestations can occur in the liver, spleen, central nervous system, heart, upper respiratory tract and bones. Cutaneous lesions of sarcoidosis may be specific, showing histopathologically sarcoid granulomas, or non‐specific, mainly erythema nodosum. Cutaneous lesions are frequently the presentation of the disease and skin biopsy enables early diagnosis. Moreover, some types of lesions have prognostic significance and may help to predict the outcome of the systemic disease.
Panniculitis
Authors: Requena Luis 
Published: August 2023

AbstractThe panniculitides comprise a group of heterogeneous inflammatory diseases that involve the subcutaneous fat. Histopathological study is required for the specific diagnosis of these disorders, because different panniculitides usually show the same clinical appearance – which typically consists of erythematous nodules on the lower extremities. However, the histopathological study of panniculitis is difficult because of the poor correlation between clinical and pathological findings and the changing microscopic appearances as the lesions evolve. Furthermore, large scalpel incisional biopsies are required in order to evaluate panniculitis fully. From the histopathological point of view, all panniculitides are somewhat mixed, because the inflammatory infiltrate involves both the septa and lobules. However, differentiation between a predominantly septal and a predominantly lobular panniculitis is usually straightforward at scanning magnification on the basis of which subcutaneous structures are the more intensely involved by the inflammatory infiltrate.
Other Acquired Disorders of Subcutaneous Fat
Authors: Chen Amy Y.‐Y. , Garg Amit 
Published: August 2023

AbstractThis chapter addresses principally non‐inflammatory acquired disorders of subcutaneous fat with an emphasis on acquired lipodystrophy, fat hypertrophy, subcutaneous lipomatosis and lipoedema. While some of the entities discussed are very common, such as cellulite and obesity, most are much rarer. Panniculitis and genetic disorders of subcutaneous fat are addressed other chapters in the book.
Purpura
Authors: Leslie Tabi A. 
Published: August 2023

AbstractThis chapter discusses purpura and bruising, terms used to describe bleeding into the skin, which may occur as isolated phenomena or as part of a systemic disorder. Clinically, purpura can represent vasculitis affecting small vessels of the skin, or may be the minor or dominant features of a systemic vasculitis. Many platelet and coagulation disorders seen by haematologists present with purpura. Purpuric lesions may be seen in disorders such as vasculitis, presenting as urticarial lesions, nodules, ulcers, livedo and skin necrosis. These signs often occur in disorders where vessels are occluded. The epidemiology, pathophysiology, clinical features, differential diagnosis and management of purpura and bruising due to different causes are discussed.
Ulceration Resulting from Disorders of the Veins and Arteries
Authors: Hafner Jürg 
Published: August 2023

AbstractLeg ulcers represent a serious burden of disease with an impact, both physical and psychological, that is underestimated. Approximately 50% of all leg ulcers are venous, and another 20% mixed. Venous leg ulcers (VLUs) represent the most advanced grade of chronic venous insufficiency (CVI). CVI results from chronic peripheral venous hypertension. Compression therapy – either using bandages or stockings – is the mainstay in the treatment of CVI and VLUs. Approximately half of patients with VLUs suffer from superficial venous reflux, which can be surgically abolished by a variety of methods. A mixed leg ulcer (MLU) is a VLU in a leg with peripheral arterial disease. MLUs are hard to heal. Management focuses initially on improving arterial inflow, mostly by the use of balloon catheter angioplasty, thereby transforming the MLU into a VLU. As soon as the arterial inflow is restored, management follows the algorithm for VLUs. Arterial leg ulcers result from local skin ischaemia in peripheral arterial disease. Management mainly consists of revascularization and skin grafting. Hypertensive ischaemic leg ulcers represent a form of skin infarction due to occlusive subcutaneous arteriolosclerosis. All patients have hypertension, and 60% have diabetes type 2. From the clinical aspect, these leg ulcers can easily be confused with pyoderma gangrenosum or vasculitic skin necrosis. Treatment consists of necrosectomy, negative pressure wound therapy and, eventually, skin grafting.
Disorders of the Lymphatic Vessels
Authors: Mortimer Peter S. , Gordon Kristiana 
Published: August 2023

AbstractLymphatic dysfunction interferes with fluid homeostasis, tissue immunity and peripheral fat mobilization. Any chronic oedema represents lymphatic failure. If impaired lymph drainage is solely responsible, then lymphoedema results. This produces characteristic skin changes known as elephantiasis as well as increased fat deposition in the subcutaneous tissues. Impaired immune cell trafficking results in an increased risk of infection, particularly cellulitis/erysipelas, which often becomes recurrent. This chapter describes the clinical consequences of lymphatic dysfunction and in particular the impact on the skin and subcutaneous tissues. The sections are divided according to common clinical presentations.
Flushing and Blushing
Authors: Raghallaigh Síona Ní , Powell Frank C. 
Published: August 2023

Abstract
Flushing and blushing are the result of transient cutaneous vasodilatation that is usually physiological in nature. A blush signifies a psychosocial response to an experienced emotion, whereas a flush is a thermoregulatory response to increased body temperature. Frequent blushing can be associated with considerable anxiety and, in some cases, social phobia. Excessive flushing may be associated with topical agents, food or alcohol intake, or rarely with underlying systemic disease. Clinical features useful in defining the diagnosis include the environmental setting in which the vascular reaction occurs, the extent and pattern of cutaneous involvement, and the presence of associated systemic symptoms. If an underlying systemic disorder is suspected, relevant investigations may be required. Understanding of the mechanisms by which these cutaneous reactions occur has increased, but treatment options remain limited.
Dermatoses of the Male Genitalia
Authors: Bunker Christopher B. , Porter William M. 
Published: August 2023

AbstractA number of common skin diseases affect the genital and genito‐crural skin only incidentally, and may present in these areas with unusual features. These are dealt with briefly or by cross‐reference to their full description elsewhere. However, those conditions that are entirely or predominantly confined to these regions are discussed in detail.
Dermatoses of Perineal and Perianal Skin
Authors: Mallon Eleanor 
Published: August 2023

Abstract
Dermatologists should be able to assess perineal and perianal skin competently and know when to involve other disciplines. The skin of the perineum includes perianal, groin and scrotum or vulval skin. Common dermatoses easily recognized elsewhere may have a different appearance in the perineum as clinical features can be altered by the microenvironment that exists in the flexures due to heat, moisture and friction. It is important to be familiar with the cutaneous manifestations of intestinal and sexually transmitted diseases. Cutaneous features of conditions such as Crohn disease or tuberculosis are relatively non‐specific and diagnosis of these diseases is reached by demonstration of their typical histology. This chapter concentrates on important entities relevant to perineal and perianal skin including pruritus ani, anal fistula, haemorrhoids, human papillomavirus infection, anal intraepithelial neoplasia and anal cancer.
Dermatoses of the Neonate
Authors: Paige David G. 
Published: August 2023

AbstractRashes in the first 4 weeks of life are common and can be alarming for both parents and medical staff. Many of these are entirely innocent and resolve spontaneously and reassurance of the parents is all that is required. It is important to be aware of such ‘benign’ rashes (as well as the appearance of preterm and post‐term skin) when assessing genuine pathology in the neonate. Pathological dermatoses may arise by a number of different mechanisms. Iatrogenic causes (medical procedures) can cause rashes/lesions bothin uteroand in the neonatal period. Transplacental transfer of antibodies, infection or malignant cells from mother to child is another mechanism for causing neonatal rashes. Infection can also be passed on from attendant medical staff. Finally, primary dermatoses may present early in the neonatal period, especially genodermatoses such as collodion baby and epidermolysis bullosa.
Dermatoses and Haemangiomas of Infancy
Authors: Higgins Elisabeth M. , Glover Mary T. 
Published: August 2023

AbstractAn overview of the most common or important dermatoses occurring in the first year of life is presented, under the categories of inflammatory, infective, reactive, developmental/genetic and miscellaneous. Emphasis is given to the key features relative to this age group, and more detailed accounts of specific disorders are found in the specialist sections elsewhere in these volumes. A section on infantile and congenital haemangiomas is also included.
Benign Cutaneous Adverse Reactions to Drugs
Authors: Ardern‐Jones Michael R. , Lee Haur Yueh 
Published: August 2023

AbstractAdverse drug reactions commonly involve the skin and represent a significant proportion of all drug‐induced illnesses. There is a broad spectrum of phenotypes encountered in cutaneous adverse reactions and many can mimic other inflammatory rashes. Rarely, drug hypersensitivity dermatoses can be life threatening and involve internal organs; however, most eruptions are mild, affect the skin only and are self‐limiting on drug withdrawal. These disorders can be considered as the benign cutaneous adverse reactions.
Severe Cutaneous Adverse Reactions to Drugs
Authors: Walsh Sarah , Yueh Lee Haur , Creamer Daniel 
Published: August 2023

AbstractSevere life‐threatening cutaneous drug reactions are rare phenomena, and may be clinically heterogeneous. They may be broadly categorized as Stevens–Johnson syndrome, toxic epidermal necrolysis, drug reaction with eosinophilia and systemic symptoms, acute generalized exanthematous pustulosis and drug‐induced generalized exfoliative dermatitis. Each has distinct clinical features, and is associated with systemic disturbance. Overlap syndromes with clinical features of more than one diagnosis are described.
Cutaneous Side Effects of Chemotherapy and Radiotherapy
Authors: Fearfield Louise , Natkunarajah Janakan 
Published: August 2023

AbstractChemotherapy drugs are a well‐recognized cause of iatrogenic injury to the skin. Antineoplastic agents generally target rapidly dividing cells and are toxic to organ systems with high proliferative activity, including the skin and its appendages. These adverse effects include toxic erythema, alopecia, hyperpigmentation, nail changes, photosensitivity and papulopustular eruptions. Recently, the emergence of novel targeted agents has led to new patterns of cutaneous toxicity. Occasionally, severe chemotherapy reactions occur, necessitating cessation of the drug, however most dermatoses can be managed by simple measures allowing patients to continue essential anticancer treatment. Radiotherapy‐associated skin side effects are common and can be divided into those occurring early (days to weeks) during the treatment and those occurring late (months to years). Most reactions are due to radiation‐induced dermatitis and are limited to the treated area, however rare skin side effects can occur distant from the radiotherapy‐treated zone.
Pressure Injury and Pressure Ulcers
Authors: Evans Robyn , Ott Carol , Reddy Madhuri 
Published: August 2023

AbstractPressure ulcers represent a significant health concern for patients, families and funding agencies. Pressure ulcers are known to increase the length of stay in acute care hospitals and may contribute to premature death. Pressure ulcers are largely preventable with the appropriate assessment and management of the various intrinsic (patient related) and extrinsic (pressure, shear, friction, skin microclimate) factors. They are classified by the extent of damage to the underlying tissue using the National Pressure Ulcer Advisory Panel staging system. There are many choices of support surfaces for the prevention and treatment of pressure ulcers; however, the challenge is to use the most cost‐effective surface given the available evidence. Infection in a pressure ulcer is one of the leading causes of infections in nursing homes and significantly delays healing. Detection of infection in pressure ulcers is challenging as the classic signs and symptoms may be absent in this chronic type of wound.
Cutaneous Photosensitivity Diseases
Authors: Ibbotson Sally , Dawe Robert 
Published: August 2023

AbstractThe photodermatoses are a diverse group of diseases, some of which, such as polymorphic light eruption, are very common and others, such as hydroa vacciniforme or xeroderma pigmentosum, are rare. It is essential not to miss the diagnosis of these conditions and referral to a specialist photodiagnostic centre is advised for many patients. Management depends on the diagnosis but includes prevention and disease suppression. Photoprotection is a mainstay approach for the photodermatoses but is often not sufficient for disease control. Desensitization using photo(chemo)therapy regimens may be feasible and systemic therapies are sometimes needed. Photosensitivity, particularly if chronic and severe, can be associated with significant psychological morbidity, which must be considered. Awareness of the possibility of vitamin D deficiency is also required.
Allergic Contact Dermatitis
Authors: Wilkinson Mark , Orton David 
Published: August 2023

AbstractAllergic contact dermatitis is the classic delayed hypersensitivity reaction, developing in two phases – sensitization and elicitation. It can have profound effects on affected individuals and may result in sickness, absence from work and increased health expense. Its diagnosis requires consideration of many factors including the patient's history and a physical examination; knowledge of the skin's reaction to various chemicals is also desirable. Above all, it requires a high degree of suspicion on the part of the clinician and tenacity, skill and experience in the investigator. The diagnosis can only be confirmed by interpreting patch test results and the technique in itself has many pitfalls for the unwary. Any sensitization identified needs to be put into the context of a past or present dermatitis, and explanations for allergen avoidance or substitution need to be provided for the patient.
Irritant Contact Dermatitis
Authors: White Jonathan M. L. 
Published: August 2023

AbstractIrritant contact dermatitis results from a non‐immune specific response to chemical and physical irritants. It is caused or exacerbated by genetic mutations (especially the filaggrin gene) and environmental factors such as temperature, air flow, low humidity and occlusion. These variables at least partly explain the great interindividual variability in irritant reactions. Reactions vary from subjective stinging reactions, mild skin dryness and redness, to potentially life‐threatening chemical burns. Dermatitis of the dorsum of the hand and interdigital space is commonly caused by an irritant dermatitis, although many different clinical patterns may be seen due to different exposures. Patch testing is mandatory to exclude an allergic contact dermatitis. Treatment is by primary prevention, avoidance/substitution of the known irritant (including occupational re‐deployment) and supportive measures. Personal protective equipment must be chosen carefully, with good knowledge of the chemical properties of the relevant irritants.
Occupational Dermatology
Authors: White Jonathan M. L. 
Published: August 2023

AbstractOccupational skin disease is best described as ‘a skin disease, which would not have occurred if the patient had not been doing the work of that occupation’. Definite proof may be difficult, but the following are suggestive: (i) occupational contact with an agent that caused similar skin changes in others; (ii) similar dermatoses in fellow workers; (iii) consistent chronology between the exposure and the disease; (iv) distribution and morphology consistent with information of exposure, and similar to other cases; and (v) confirmatory test results. Legal definitions vary from country to country, as does the amount of compensation offered. Certain occupations are at high risk of developing skin problems, such as hairdressers, medical staff and construction workers. Irritant contact dermatitis is the commonest work‐related skin condition. Thorough history, examination, investigations and site visits with chemical testing may be required to make a secure diagnosis.