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An 11-month-old presents with generalized 
rash, scattered blisters

An 11-month-old female is admitted to the PICU for evaluation and treatment of a severe rash and subjective fever. The history of the chief complaint began 4 days earlier with some fussiness and the appearance of some erythema on her cheeks and puffiness of her eye lids. The next day, the rash had spread to her body and extremities, and she was taken to a local ED, where she was given some oral antihistamine and prednisone for an allergic reaction. She returned to the ED the next day with further progression of the rash and was treated with a dose of IV steroids and diphenhydramine hydrochloride (Benadryl, McNeil Consumer Healthcare). By the next day, her condition had worsened with the development of some desquamation and numerous small blisters, and she was referred for admission with the diagnosis of Stevens-Johnson syndrome vs. toxic epidermal necrolysis.

Her past medical history was that of a previously healthy child who was well and on no medications prior to the onset of the chief complaint. Her immunizations were up-to-date and had no known sick contacts. She had no known allergies, and there has been no recent travel or animal exposure.

James H. Brien

Examination revealed a fussy 11-month-old female with a low-grade fever and otherwise normal vital signs, along with a bright red, very painful generalized rash, with areas of desquamation and some scattered, small blisters (Figures 1-3). She also had copious mucopurulent drainage from her nose and eyelids, but her eyes and all mucous membrane surfaces were clear (Figure 4). She had no lymphadenopathy or any other abnormal findings.

Examination revealed a fussy 11-month-old female with a bright red, very painful generalized rash, with areas of desquamation and some scattered, small blisters (Figures 1-3). She also had copious mucopurulent drainage from her nose and eyelids, but her eyes and all mucous membrane surfaces were clear (Figure 4).

She had numerous admission lab tests, including a normal CBC, complete metabolic profile and urine analysis. Blood and urine cultures are pending, along with Mycoplasma titers.

What’s Your Diagnosis?

A. Stevens-Johnson syndrome (SJS)

B. Staphylococcal scalded skin syndrome (SSSS)

C. Toxic epidermal necrolysis (TEN)

D. Staphylococcal scarlet fever (SSF)

The diagnosis I was taught in fellowship 31 years ago by Jim Bass, MD, was SSF (D). But don’t feel bad if you chose SSSS, as many experts still consider SSF and SSSS to be part of the same spectrum of staph toxin-mediated diseases. However, there’s clearly a difference in the dramatic dermatologic consequences of SSSS and that of SSF.

The data seem to favor SSSS being caused by a Staphylococcus aureus that releases the exotoxins, exfoliatin A or B into the bloodstream, which has a disruptive effect on the intracellular bonds within the granular layer of the epidermis, thereby causing the development of very large, thin and flaccid bullae. This blistering appearance is where SSSS gets its name. Additionally, some researchers have found that most cases of SSF are found to be associated with staph enterotoxin B, which closely resembles the same protein that is associated with streptococcal SF (streptococcal pyrogenic exotoxin type A). And, considering that the rash of staphylococcal SF more closely resembles streptococcal SF than SSSS, many experts do not consider SSF to be in the same spectrum as SSSS. The debate will probably continue for some time to come, but I’m sold until there’s more proof to the contrary.

Patients respond dramatically to IV hydration and appropriate anti-staph antimicrobial therapy, as shown in this patient just 24 hours later in Figures 5 and 6.

The key elements of SSF include: 1) the painful erythroderma with desquamation and small blisters in the acute stage; 2) a lack of mucous membrane involvement; and 3) the copious discharge from the nose and eyelids. The patient may present with or without fever. They respond dramatically to IV hydration and appropriate anti-staph antimicrobial therapy, as shown in this patient just 24 hours later in Figures 5 and 6.

I would recommend treating for MRSA until culture results are available. Five days after admission to the PICU, she was discharged home on oral cephalexin for the MSSA recovered from her nose, where a high inoculum of the organism can be found. You have to remember to ask the lab for culture and sensitivities, not just an MRSA screen, like many hospitals do for infection control purposes. The nose will virtually always yield the 
S. aureus responsible for this condition. The eyelids also are sometimes culture-positive, but the nose is where the action is unless there’s an infected wound or other site somewhere else. As expected, in the patient described in this case, all other cultures and studies were negative.

SJS and TEN are two more conditions that are often thought to be part of the same spectrum of diseases, with TEN being at the most severe end. There is now consensus among academic dermatologists on this. However, others believe these conditions are part of the erythema multiforme spectrum, with erythema multiforme major being essentially the same as SJS. However, that debate is for another time in another column.

SJS by definition is an inflammatory skin condition that involves at least two mucous membrane surfaces. In my experience, SJS typically is triggered by a drug, such as a sulfa-containing or other antibiotics, anticonvulsant, or infections with herpes simplex virus or Mycoplasma. Some experts feel that herpes simplex is the trigger for the vast majority of cases. In any case, it typically begins as a morbilliform rash with lesions that become more numerous and confluent, and within a few days is widespread with small blisters, along with necrotic mucous membrane lesions (Figure 7). TEN, however, being much more severe, often resembles a burn patient with widespread subepidermal injury. The cases I have seen were associated with either sulfa or anticonvulsant usage, although other medications also have been implicated.

Morbilliform rash with small blisters, along with necrotic mucous membrane lesions.

Even if there are close similarities in the rash of SJS with SSF in a given patient, the mucous membrane involvement of SJS makes differentiation relatively easy. Treatment for SJS and TEN is support as needed, which usually means a transfer to a burn unit for TEN. They also should be seen by ophthalmology to rule out eye involvement. Steroids have not been shown to help. Obviously, the offending drug should be stopped as soon as possible.

By the way, many of us believe SSF to be the same entity as fourth disease, based on the papers published by Martin E. Weisse, MD, in The Lancet in January 2001, and Keith Powell, MD, in AJDC in January 1979. We may never know for sure, but until a better argument can be made, I am on board with them being the same disease.

Disclosure: Brien reports no relevant financial disclosures.