The generalized form of SSSS is termed pemphigus neonatorum or Ritter’s disease. Risk
factors for development in adults include renal dysfunction, lymphoma, and immunosup-
pression (112,119,120). Patients with pemphigus neonatorum present with fever, erythema,
malaise, and irritability. They then develop large superficial blisters that rupture easily because
of friction (112). A positive Nikolsky sign refers to dislodgement of the superficial epidermis
when gently rubbing the skin (121). If untreated, the epidermis will slough off leaving
extensive areas of denuded skin that are painful and susceptible to infection. Mucous
membranes are not affected in SSSS.
The mortality rate in children remains below 5%. Potentially fatal complications in
infants and young children occur because of the loss of protective epidermis. Hypothermia,
dehydration, and secondary infections are the leading causes of morbidity and mortality for
these age groups affected by generalized SSSS (122). The mortality for adults with generalized
SSSS is 60%, probably due to the associated comorbidities such as renal dysfunction,
immunosuppression, or malignancy found in this population (123).
Diagnosis of both generalized and localized SSSS is based on clinical characteristics. A
thorough exam looking for foci of infection (pneumonia, abscess, arthritis, endocarditis,
sinusitis, etc.) should be undertaken. Unfortunately, in most cases, no focus is ever found (112).
Blood cultures are usually negative because toxins are produced at a distant site (119,124).
A number of different tests, including PCR, enzyme-linked immunosorbent assays,
radioimmune assays, and reverse latex agglutination assays, can be used to demonstrate toxin
production byS. aureus(125). The diagnostic challenge is that bacteria must first be isolated.
When the diagnosis is uncertain, a skin biopsy may be the optimal test. The biopsy typically
reveals mid-epidermal splitting at the level of the zona granulosa without cytolysis, necrosis,
or inflammation (126). Staphylococci may be seen in bullous lesions of localized disease, but
are rarely seen in the bullous lesions of generalized disease (120).
Scarlet Fever
Scarlet fever is the result of infection with aStreptococcus pyogenesstrain (i.e., group A
streptococcus) that produces a pyrogenic exotoxin (erythrogenic toxin). There are three
different toxins, types A, B, and C, which are produced by 90% of these strains. Scarlet fever
follows an acute infection of the pharynx/tonsils or skin (8). It is most common in children
between the ages of 1 and 10 years (111).
The rash of scarlet fever starts on the head and neck, followed by progression to the
trunk and then extremities (8,127). The rash is erythematous and diffuse and blanches with
pressure. There are numerous papular areas in the rash that produce a sandpaper-type quality.
On the antecubital fossa and axillary folds, the rash has a linear petechial character referred to
as Pastia’s lines (127). The rash varies in intensity but usually fades in four to five days. Diffuse
desquamation occurs after the rash fades (127). Diagnosis of scarlet fever can usually be made
on a clinical basis. Confirmation of the diagnosis is supported by isolation of group A
streptococci from the pharynx and serologies (111).
Kawasaki Disease
Kawasaki disease (KD) is an acute, self-limited, systemic vasculitis of childhood (128–130). KD
was first described by Tomisaku Kawasaki in Japan in 1961 (128) and is the predominant cause
of pediatric-acquired heart disease in developed countries (130). The signs and symptoms
evolve over the first 10 days of illness and then gradually resolve spontaneously in most
children. The diagnostic criteria for classical KD include the following (128):
- Fever for five days or more that does not remit with antibiotics and is often resistant
to antipyretics. - Presence of at least four of the following conditions:
a. Bilateral (nonpurulent) conjunctivitis
b. Polymorphous rash
c. Changes in the lips and mouth: reddened, dry, or cracked lips; strawberry tongue;
diffuse erythema of oral or pharyngeal mucosa
Fever and Rash in Critical Care 35