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  • Erythema nodosum (EN) is an acute panniculitis consisting of deep, painful, bilateral erythematous nodules, usually on the lower extremities, resulting from an underlying systemic illness.

  • Erythema multiforme (EM) is an acute, immune-mediated mucocutaneous condition characterized by target lesions with concentric zones of color change, most commonly associated with herpes simplex virus (HSV) infection.

  • Stevens–Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe immune-mediated hypersensitivity reactions characterized by diffuse bullous lesions and mucocutaneous involvement most commonly precipitated by medications such as anticonvulsants.

  • Staphylococcal scalded skin syndrome (SSSS) is characterized by an erythematous rash followed by diffuse epidermal exfoliation.

  • Purpura fulminans is a severe form of rapidly progressive purpura with multiorgan failure, usually associated with meningococcemia.

  • Nonaccidental trauma should be suspected if bruising occurs on non-bony prominences or in areas not normally subjected to trauma during routine child play.

  • Toxic shock syndrome (TSS) is an acute, toxin-mediated illness characterized by fever, erythroderma, hypotension, multiorgan involvement, and desquamation. Streptococcal TSS (STSS) is a similar disease caused by invasive group A Streptococcus (GAS). Each of these is a shock state.

  • Necrotizing fasciitis (NF) is a rapidly progressive, life- and limb-threatening infection by virulent bacteria with severe inflammation of the fascia and surrounding subcutaneous tissues.

  • Kawasaki disease (KD) is an acute, self-limited, vasculitic syndrome in children. The diagnosis is established clinically by the presence of prolonged fever and four of the following five clinical features: conjunctival injection, oropharynx erythema, cervical lymphadenopathy, hand and foot erythema/swelling, and rash.

  • Urticaria, often referred to as hives, appears as blanchable, pruritic, raised, well-circumscribed areas of edema and erythema involving the epidermis and dermis.

  • Angioneurotic edema (AE) is a self-limited localized swelling due to extravasation of fluid into interstitial tissues, most commonly involving the head, neck, hand, and gastrointestinal tract. Laryngeal involvement is life-threatening.

Dermatologic signs of systemic disease are the primary focus of this chapter. Many diseases have clinical presentations where the dermatologic manifestations play a role in helping the clinician make the underlying diagnosis. Table 95-1 is a list of systemic diseases with their corresponding dermatologic manifestations, and the sections below describe pediatric emergencies in which the dermatologic characteristics play a large role in diagnosis.

TABLE 95-1Characteristic Dermatologic Manifestations of Systemic Diseases

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