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Cutaneous lymphoproliferative diseases and related disorders

Classification of lymphomas The classification of lymphomas and hematopoietic neoplasms has undergone radical changes in philosophy and diagnostic criteria since the early days of Lukes and Collins, Rappaport, and the subsequent Updated Kiel classification and the Working Formulation. The majority of these earlier classifications relied mainly on morphology to differentiate between entities, and were designed to be applicable to nodal disease with little or no attention…

Sentinel lymph node biopsies

Introduction This chapter is based on the author's previous publications on this topic. Management of patients with primary melanoma in vertical growth phase who have no evidence of regional nodal metastases on palpation or ultrasound has been controversial for many years. Until recently, the treatment options were: observation with lymphadenectomy delayed until regional metastases became clinically evident (watch and wait), elective (prophylactic) regional lymph node dissection…

Tumors of the conjunctiva

Introduction Anatomy and histology of the conjunctiva The conjunctiva is a mucous membrane that covers the surface of the eyeball and posterior aspect of the eyelid that functions to protect the eye and allow the eyelids to move smoothly over the globe ( Fig. 27.1 ) . It is divided into four main regions: limbus, bulbar, fornix, and palpebral (tarsal) conjunctiva. The limbus ( Fig. 27.2…

Melanoma

Melanoma Clinical features Over the last several decades, the conspicuous increasing incidence of this malignant neoplasm has made this disease more prominent, linked to some rise in morbidity and mortality. While roughly half of the major cancers in the United States showed decreasing incidence trends from 2009 to 2013, melanoma incidence continued to significantly increase over this period just as it had done previously in both…

Melanocytic nevi

Ephelide Clinical features Ephelides (freckles) are extremely common lesions that present as clusters of small (approximately 2.0 mm in diameter), uniformly pigmented macules ( Fig. 25.1 ). They are directly related to exposure to sunlight and are much more conspicuous in summer than in winter. Sites of predilection therefore include the nose, cheeks, shoulders, and dorsal aspects of the hands and arms. Although virtually everyone shows some…

Tumors of the surface epithelium

Epidermal nevi Clinical features Epidermal nevi are of the following three subtypes, which are histologically identical and differ only in the degree of clinical involvement: nevus verrucosus, nevus unius lateris, ichthyosis hystrix (this has no relationship to ichthyosis). Lesions, which may be present at birth or develop during childhood, are usually yellowish-brown warty papules or plaques with irregular margins. They commonly affect the trunk or limbs…

Diseases of the nails

Introduction Nail histopathology requires a sound knowledge of the anatomy of the nail apparatus and excellent clinical-pathological correlation. The first major challenge for the nail histopathologist is to obtain interpretable biopsies, i.e., specimens that are correctly sampled by the nail surgeon, but also that are correctly handled and processed in the pathology laboratory. This requires input from the physician, who is expected to provide high-quality clinical…

Diseases of the hair

Introduction A distinctive phenomenon of the human evolutionary process is the loss of a great proportion of the body hair. Vestiges of hair remain in areas such as the scalp, axillae, genital areas, and face. Among the many species of primates, only humans are almost hairless. The time and cause of this remarkable transformation remains an enigma. Whatever the explanation for the loss of hair in…

Diseases of collagen and elastic tissue

Introduction Improved and detailed understanding of the molecular basis of many of the diseases in this chapter continues to progress. While Ehlers-Danlos has been well understood for many years, our understanding has further expanded and a new classification scheme systematizes by both clinical presentation and pathogenesis into multiple categories. In other diseases, we have gone from virtually no knowledge or only speculation regarding their molecular determinates…

Disorders of pigmentation

Disorders of Hypopigmentation Vitiligo Clinical features Vitiligo is a common acquired disease of unknown etiology characterized by loss of melanocytes resulting in macular areas of leukoderma that progressively enlarge and often become confluent. The incidence has been calculated as between 1% and 2% of the population. It affects all races but appears to be more common in people with dark skin. However, the latter may represent…

Human immunodeficiency virus (HIV) and acquired immunodeficiency syndrome (AIDS)-associated cutaneous diseases

Introduction UNAIDS estimates that globally 36.7 million people, including 2.1 million children, were living with human immunodeficiency virus (HIV) infection in 2016. The number of people who acquired HIV infection in 2016 was 1.8 million. Since the start of the epidemic, 35 million people have died of acquired immune deficiency syndrome (AIDS)-related illnesses. Tuberculosis is still the leading cause of death in people living with HIV;…

Infectious diseases of the skin

Viral Infections Common wart The common wart (verruca vulgaris) is caused by infection with human papillomavirus (HPV) ( Fig. 18.1 ). HPV is a DNA virus of the papovavirus family. The number of known HPV genotypes currently stands at more than 200, classified according to the extent of their DNA homology (DNA hybridization) ( Table 18.1 ). In order for an HPV type to be regarded…

Idiopathic connective tissue disorders

Lupus erythematosus Lupus erythematosus is a complex disorder associated with numerous clinical signs and symptoms and a wide range of laboratory abnormalities. It shows a spectrum of varying prognoses, ranging from a benign, solely cutaneous variant (localized discoid) through to a potentially fatal systemic illness. The range of subtypes is shown in Table 17.1 . Table 17.1 Lupus erythematosus: subtypes Discoid lupus erythematosus (localized) Discoid lupus…

Vascular diseases

Introduction Vasculitis and other forms of vascular damage are the subjects of this chapter. Although minimal criteria for the diagnosis of vasculitis may differ among experts, the presence of inflammation and some evidence of vascular damage in the form of vessel wall/endothelial cell necrosis or fibrinoid change fulfill most authorities' criteria for a diagnosis of vasculitis. Some, however, apply the term less restrictively to vascular inflammation…

Neutrophilic and eosinophilic dermatoses

Pyoderma gangrenosum Clinical features Pyoderma gangrenosum is an uncommon disease of obscure etiology. It appears to be somewhat more common in women and, although it may occur at any age, most patients are in their fourth or fifth decade. Presentation in children is uncommon, but it has been seen even in infants, and rare familial cases have been documented. The disease may also present in pregnancy,…

Cutaneous adverse reactions to drugs

Adverse Drug Reactions – Introduction Adverse drug reactions are unintended and undesired effects of drugs used for prevention, diagnosis, or treatment of disease. In light of the ever-increasing number of medications available, it should come as no surprise that such reactions are extremely common. The incidence statistics vary considerably depending upon the method by which the data are derived and the nature of the population under…

Degenerative and metabolic diseases

The hyperlipidemias The hyperlipidemias may present as cutaneous xanthomata, which are localized aggregates of histiocytes containing accumulated lipid (primarily free and esterified cholesterol), in the form of five main clinical types: eruptive, tendinous, tuberous, planar, disseminated. The last, xanthoma disseminatum, in which serum lipid levels are normal, is discussed in Chapter 29 (see xanthogranuloma). Xanthoma cells are histiocytes and express CD4, CD11c, CD14b, CD68 and CD163…

Diseases of the anogenital skin

Introduction This section concerns itself principally with dermatological disorders specific to the anogenital skin. Many of the dermatological conditions that present in the skin elsewhere sometimes affect the anogenital area although this site may be one of predilection. Clinical and histologic features can be modified by the chronicity of the problem, treatments applied, and the occlusive effect of this natural flexural site. Over the years, various…

Diseases of the oral mucosa

Introduction Oral and maxillofacial pathology is the specialty of dentistry that is involved in the histopathological and clinical diagnosis, as well as management of diseases of the oral mucosa and supporting bone and soft tissues, teeth, salivary glands, lip vermilion, and perioral skin. It would be impossible to discuss diseases affecting all of the above entities in one chapter. As such, this chapter is confined to…

Inflammatory diseases of the subcutaneous fat

Inflammatory diseases of the subcutaneous fat are a source of considerable confusion and often cause diagnostic difficulty to clinicians and pathologists alike. This stems in part from the use of classifications and clinical descriptions based on time-honored but outdated literature. Inadequate biopsy specimens are also a source of considerable difficulty, particularly the punch biopsy specimen, which often yields no subcutaneous fat at all. Similarly, histologic subdivision…