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Vesiculopustular and Erosive Disorders in Newborns and Infants

A wide variety of conditions can cause vesicles, pustules, bullae, erosions, and ulcerations during the newborn period and infancy. Accurate and prompt diagnosis is important because some of the underlying disorders represent potentially life-threatening infections; conversely, many are benign and self-limited. Therefore, it is essential to develop a systematic approach to the evaluation and treatment of newborns and infants with these types of skin lesions. An…

Other Vesiculobullous Diseases

Vesiculobullous diseases of the skin encompass a wide range of entities, including autoimmune bullous diseases and inherited blistering disorders (see 28, 29, 30, 31, 32 ). This chapter focuses on the remaining non-infectious disorders in which cutaneous bullae develop and represent the primary clinical manifestation. Bullosis Diabeticorum Synonyms ▪ Diabetic bullae ▪ Bullous eruption of diabetes mellitus Key features ▪ A rare condition associated with diabetes…

Epidermolysis Bullosa

Synonyms ▪ All forms of EB: EB hereditaria ▪ EB simplex: epidermolytic EB ▪ Junctional EB: EB atrophicans, EB letalis ▪ Dystrophic EB: EB dystrophica Key features ▪ Epidermolysis bullosa (EB) encompasses multiple clinically distinctive disorders that share three major features: genetic transmission, mechanical fragility of the skin, and blister formation ▪ There are four major forms of inherited EB – EB simplex, junctional EB, dystrophic…

Dermatitis Herpetiformis and Linear IgA Bullous Dermatosis

Key features ▪ Dermatitis herpetiformis (DH) is differentiated from linear IgA bullous dermatosis (LABD) on the basis of granular IgA versus linear IgA deposition at the basement membrane zone, as observed by direct immunofluorescence ▪ DH is a cutaneous manifestation of celiac disease (CD) and is associated with gluten sensitivity in virtually all cases ▪ DH and CD are genetic disorders strongly associated with the HLA-DQ2…

Pemphigoid Group

Bullous Pemphigoid Synonym ▪ Pemphigoid Key features ▪ Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease, and its onset is often after 60 years of age ▪ It is usually a chronic disease, with spontaneous exacerbations and remissions, which may be accompanied by significant morbidity ▪ BP is associated with tissue-bound and circulating autoantibodies directed against BP antigen 180 (BP180, BPAG2 or type…

Pemphigus

Synonyms ▪ Fogo selvagem: endemic pemphigus foliaceus; Brazilian pemphigus ▪ Pemphigus erythematosus: Senear–Usher syndrome ▪ IgA pemphigus: intercellular IgA dermatosis; intraepidermal neutrophilic IgA dermatosis; intercellular IgA vesiculopustular dermatosis ▪ Paraneoplastic pemphigus: paraneoplastic autoimmune multi-organ syndrome Key features ▪ Pemphigus is a group of autoimmune blistering diseases of the skin and mucous membranes that is characterized by: histologically, intraepidermal blisters due to the loss of cell–cell adhesion…

The Biology of the Basement Membrane

Synonyms ▪ Bullous pemphigoid antigen 1: BP230 ▪ Bullous pemphigoid antigen 2: BP180; type XVII collagen ▪ Laminin 311: laminin 6 ▪ Laminin 332: laminin 5; epiligrin; kalinin; nicein; GB3 antigen; BM600 ▪ Laminin 511: laminin 10 ▪ Type VII collagen: the epidermolysis bullosa acquisita antigen ▪ Nidogen: entactin Key features ▪ Basement membranes serve as: (1) a substrate for attachment of cells; (2) a template…

Pregnancy Dermatoses

For decades, the specific dermatoses of pregnancy represented a confusing group of overlapping entities, understood largely through anecdotes and case reports. However, more recent reviews have simplified and condensed the list of pregnancy-specific dermatoses ( Table 27.1 ) . For example, impetigo herpetiformis is now generally recognized as pustular psoriasis, perhaps induced by the relative hypocalcemia of pregnancy (see Ch. 8 ). Also, prurigo annularis, first…

Neutrophilic Dermatoses

The neutrophilic dermatoses constitute a heterogeneous but linked spectrum of diseases , with significant overlapping histopathologic findings and similar pathogenic mechanisms and therapeutic approaches. They are often associated with underlying internal diseases, which may have significant morbidity and mortality. Histologically, these disorders are characterized by perivascular and diffuse neutrophilic infiltrates without any identifiable infectious agents ( Fig. 26.1 ). The cutaneous manifestations vary, from urticarial plaques…

Eosinophil-Associated Dermatoses

Key features ▪ Eosinophil infiltration of the skin is commonly observed and therefore clinicopathologic correlation is required to determine a correct diagnosis ▪ Eosinophil-associated dermatoses include arthropod bite reactions and drug eruptions (“bugs and drugs”), as well as parasitic infections, autoimmune blistering diseases, and Wells syndrome ▪ Eosinophils commonly disrupt and lose their morphologic integrity as they deposit toxic granule proteins and other inflammatory mediators in…

Cutaneous Vasculitis

Key features ■ Cutaneous signs of vasculitis are a reflection of the size of the vessels involved ■ Vasculitis can be limited to the small vessels of the skin or it can be a sign of life-threatening internal organ involvement ■ The clinical diagnosis of cutaneous vasculitis requires histopathologic confirmation, and multiple biopsies may be required Introduction Vasculitis represents a specific pattern of inflammation of the…

Cutaneous Manifestations of Microvascular Occlusion Syndromes

Introduction The differential diagnosis of microvascular syndromes involving the skin is extensive and it is critical to distinguish between inflammatory injury and non-inflammatory occlusive injury to vessels. The diagnosis of occlusive syndromes in the skin is greatly facilitated by recognizing the telltale lesions of retiform purpura or non-inflammatory (bland) necrosis. Diseases that produce skin lesions secondary to microvascular occlusion have often been lumped with cutaneous vasculitic…

Purpura: Mechanisms and Differential Diagnosis

Purpura Introduction The differential diagnosis of purpura is extensive, but the evaluation of a patient with purpura can be greatly facilitated by categorizing purpuric lesions into morphologic subsets . The number of lesions and their distribution pattern should also be assessed, including whether all the lesions are purpuric or just those located on the distal lower extremities. This chapter serves as an introduction to a method…

Environmental Causes of Dermatitis

Plant Dermatitis Joao Paulo Niemeyer-Corbellini Omar Lupi Laila Klotz Livia Montelo Synonym Plant dermatitis Key Points Plant dermatitis may be produced by different plant species. It is increasing in incidence worldwide. It may be classified as mechanical injury, primary irritant (toxic) dermatitis, allergic phytodermatitis, and contact urticaria. Acute cases present as linear urticarial, erythematous, and blistering lesions that may leave hyperpigmented macules. Chronic cases present as lichenified, scaling dermatitis. Diagnosis…

Pigmentary Disorders

Introduction Pigmentation refers to pigment formation in tissues. Abnormal accumulation or decreased / absent pigment causes skin pigmentation disorders that present as hyperchromic (hyperpigmentation) or hypochromic / achromic (leukoderma) lesions, respectively. The disorders may be caused by genetic, metabolic, endocrine, nutritional, neoplastic, chemical, physical, inflammatory, allergic, and infectious factors. Hyperpigmentation Hyperpigmentation may be endogenous or exogenous according to the origin of the pigment. It may also be congenital / hereditary or acquired.…

Fogo Selvagem (Endemic Pemphigus Foliaceus)

Introduction The term pemphigus describes a group of autoimmune vesicobullous diseases where autoantibodies are directed against the ectodomains of the desmosomic strutures of the epidermis, culminating in keratinocyte detachment (acantholysis) and intraepithelial blister formation. Autoantibodies against stratified epithelial-specific desmosomal glycoproteins are found in all forms of pemphigus. Two of these transmembrane glycoproteins, known as desmoglein 1 (Dsg1) and desmoglein 3 (Dsg3), are recognized by pemphigus foliaceus…

Nutritional Diseases

Obesity Synonym Excess weight Key Points Overweight. Body mass index (BMI). Introduction Obesity is an excess of body fat in comparison with the lean mass of the body as a consequence of a chronic instability between energy ingestion and expenditure. Like malnutrition, obesity is part of human history. However, in the same way that malnutrition has always been connected to poverty, obesity has always been associated…

Tungiasis

Key Points The female adult flea ( Tunga penetrans ) burrows into epidermis, mostly of the feet, and grows by 2000-fold within 3–7 days. The embedded sand flea causes intense inflammation with pain and itching, resulting in acute and chronic clinical pathology, sleep disturbance and impaired mobility. Off-host stages (eggs, larvae, pupae, and adult males) develop in dry, sandy soils and floors with cracked surfaces. Tungiasis…

Myiasis

Key Points Infestation of vertebrates with dipterous larvae. Anatomic (bloodsucking, cutaneous, or cavitary) or ecological (obligatory, facultative, or accidental) classification. Cutaneous manifestations include furuncular, migratory, and wound myiasis. Dermatobia hominis and Cordylobia anthropophaga are the most common causative agents of furuncular myiasis. Clinical features distinguish migratory myiasis from helminthic cutaneous larva migrans. Mechanical extraction and surgical removal are the therapies of choice. Introduction Myiasis is the…

Pediculosis

Key Points Blood-sucking parasites specialized to certain types of human hair. Body but not hair lice transmit typhus and trench fever. Highest prevalence is in Caucasians owing to their more cylindrical hair structure. Nits are cemented to hair shafts predominantly on the retroauricular occiput. Lice combs are useful for diagnosis. Intense pruritus is common. Body and crab lice may cause maculae ceruleae. Treatment with topical permethrin,…