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Vasculitis and Other Purpuric Diseases

These diseases are all characterized by vascular damage of some type. Extravasated erythrocytes (1.40) often produce the appearance of purpura (1.120). Some of these diseases are considered to be true vasculitis. Vasculitis (literally, inflammation of the blood vessels) cannot be diagnosed with certainty unless there is visible vessel wall damage ( necrosis , hyalinization, or fibrin in the wall ) and inflammatory cells in vessel walls.…

Reactive Erythemas

The erythemas in this chapter are generally viewed as reactions to infections , drugs , malignancies , or other miscellaneous things. They are generally more dermal , having less scale or epidermal changes than the diseases in Chapter 2 . The erythema blanches , and there is no purpura or vasculitis as in Chapter 4 . When erythemas make rings, they are annular (1.5), and are…

Eczematous and Papulosquamous Diseases

The diseases in this chapter have dominant epidermal changes (clinical scaling, pathologic changes of spongiosis and parakeratosis), as opposed to rashes in 3, 4 , which primarily have dermal changes. It has been said by Levine that the diseases in this chapter are “outside jobs,” heavily influenced by external factors or contactants. Those in 3, 4 are “inside jobs” more heavily influenced by internal antigens. This…

Reactive erythema

Introduction The term reactive erythema refers to a group of disorders characterized by erythematous patches, plaques, and nodules that vary in size, shape, and distribution. Unlike other specific and intrinsic dermatoses, these represent cutaneous reaction patterns triggered by a variety of either endogenous triggers or environmental agents. In children, the most common reactive erythemas include drug eruptions, urticaria, viral exanthems, erythema multiforme (EM), erythema nodosum (EN),…

Clinical and Pathologic Findings with Differential Diagnostic Lists

The differential diagnostic lists that follow in this chapter serve as a starting point for evaluation of an unknown skin biopsy. Such lists have some advantages over algorithms because they provide multiple pathways for getting back on track toward the correct diagnosis. By contrast, some algorithms provide fatal forks in the road that, if selected incorrectly, might lead the pathologist toward a misdiagnosis based upon too…

Pigmentary disorders

Introduction Although most disorders of pigmentation in infancy and childhood are of cosmetic concern only, some provide clues to an underlying multisystem disease. Disorders of pigmentation may be differentiated clinically by the presence of either increased or decreased pigmentation (and sometimes both in the same patient!) occurring in a localized or diffuse distribution. An algorithmic approach to diagnosis for disorders of pigmentation is summarized at the…

Nodules and tumors

Introduction Nodules and tumors in the skin often raise fears of skin cancer. Fortunately, primary skin cancer is extremely rare in childhood, and most infiltrated plaques and tumors are benign ( Table 5.1 ). Hemangiomas, congenital nevi, and tumors of the newborn are reviewed in Chapter 2 , while pigmented nevi and melanomas are discussed in Chapter 6 . The focus in this chapter is disorders…

Vesiculopustular eruptions

Introduction Vesiculopustular eruptions range from benign, self-limited conditions to life-threatening diseases. Early diagnosis, especially in the young or immunocompromised child, is mandatory. An algorithmic approach to diagnosis for vesiculopustular dermatoses is summarized at the end of the chapter (see Fig. 4.29 ). An understanding of the structures that account for normal epidermal and basement membrane zone adhesion provides clues to the clinical diagnosis and pathogenesis of…

Papulosquamous eruptions

Introduction Papulosquamous eruptions comprise a group of disorders characterized by the presence of superficial papules and scale. These conditions account for a large number of patients in both pediatric dermatology and pediatric primary-care practice. In disorders of keratinization (psoriasis, pityriasis rubra pilaris, keratosis follicularis, ichthyosis, hyperkeratosis of the palms and soles, and porokeratosis), cutaneous lesions develop as a result of either genetically programmed retention or increased…

Neonatal dermatology

Introduction Newborn skin differs from adult skin in several important ways ( Table 2.1 ). It has less hair and fewer sweat and sebaceous gland secretions, is thinner, has fewer intercellular attachments, and has fewer melanosomes. These differences are magnified in the preterm neonate. As a consequence, newborns are not as well equipped to handle thermal stress and sunlight, have increased transepidermal water loss (TEWL) and…

Disorders of pigmentation

Although chiefly of cosmetic significance, disorders of pigmentation are among the most conspicuous and thus can have profound psychosocial implications for pediatric patients. The most important pigments in skin are melanin, reduced and oxygenated hemoglobin, and carotene. Melanin is a pigment produced by melanocytes, specialized dendritic cells derived from the neural crest that migrate to the basal layer of the epidermis during embryogenesis. Melanocytes synthesize and…

Introduction to pediatric dermatology

Anatomy and function of the skin Most of us think of skin as a simple, durable covering for the skeleton and internal organs. Yet skin is actually a very complex and dynamic organ consisting of many parts and appendages ( Fig. 1.1 ). The outermost layer of the epidermis, the stratum corneum, is an effective barrier to the penetration of irritants, toxins, and organisms, as well…

Histiocytoses and malignant skin diseases

The histiocytoses are a broad group of disorders characterized by an abnormal proliferation of the histiocyte, a type of progenitor cell in the bone marrow ( Box 10.1 ). Some clinically relevant types of histiocytes include the Langerhans cell, the dermal dendrocyte, and cells of mononuclear cell/macrophage lineage. Several histiocytoses are discussed in this chapter. Malignant disorders to be discussed include hematologic malignancies (including leukemia, lymphoma,…

Cutaneous tumors and tumor syndromes

Because of the increasing public awareness and incidence of skin cancer, physicians are often consulted regarding tumors of the skin. In children, the vast majority of cutaneous tumors are benign, and their importance lies predominantly in the cosmetic defect they may create or in their occasional association with systemic disease. Malignant skin lesions, however, despite their relative rarity in children, cannot be completely disregarded or ignored.…

Acne vulgaris and other disorders of the sebaceous and sweat glands

Disorders of the sebaceous glands Acne vulgaris Acne vulgaris is the most common skin problem in the United States, affecting nearly 80% to 85% of individuals at some point between 11 and 30 years of age. , Although not a serious disease, acne may be the source of permanent scarring and, even more importantly, psychosocial morbidity and decreased emotional well-being. Withdrawal from society, depression, and decreased…

Disorders of hair and nails

Hair Hair is a protein by-product of follicles distributed everywhere on the body surface except the palms, soles, vermilion portion of the lips, glans penis, penile shaft, nail beds, and sides of the fingers and toes. Although hair is of minimal functional benefit to humans, the psychologic effects of disturbances of hair growth are commonly a source of great concern to children, adolescents, and their parents.…

Hereditary disorders of the dermis

Several hereditary disorders of the skin primarily manifest as disorders of the dermis. Clinical manifestations range from laxity of skin to infiltrated papules and from rigidity to thinning of the dermis. Disorders of mucopolysaccharides are reviewed in Chapter 24 . Ehlers–danlos syndrome Ehlers–Danlos syndrome (EDS) consists of a group of six inherited disorders of collagen characterized by increased cutaneous elasticity, hyperextensibility of the joints, and fragility…

Hereditary disorders of cornification (the ichthyoses and palmoplantar keratodermas)

The hereditary disorders of cornification, or the ichthyoses, are a group of disorders characterized by scaling, skin thickening, and usually underlying inflammation, , leading to a significant impact on quality of life. In many patients, itch is also an issue. Ichthyoses are largely distinguished by their clinical and, in some cases, histologic and ultrastructural features. During the past 2 decades, next-generation sequencing has facilitated the discovery…

Papulosquamous and related disorders

Childhood psoriasis Psoriasis is a relatively common immune-mediated disorder, which occurs overall in 0.5% to 0.8% of pediatric patients, with a linear increase in prevalence by age from 0.2% at 2 years to 1.2% at 18 years. Psoriasis rarely is present at birth. , Approximately one-third of adults with psoriasis noted the onset of disease during the first 2 decades of life. As in the adult…

Eczematous eruptions in childhood

Eczematous eruptions are characterized as inflamed papules and plaques, often in association with pruritus and serous discharge. The specific subtype of eczematous dermatitis is based on the clinical morphology, distribution of lesions, and, in many cases, history of exposure. Biopsy of the skin in these conditions is usually not helpful, except to consider alternative diagnoses with distinct histopathologic features. Atopic dermatitis Atopic dermatitis (AD) is one…