November 25, 2014
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Teenage boy with suspected streptococcal pharyngitis, pneumonia and conjunctivitis

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A 13-year-old male was admitted to the hospital with right middle lobe pneumonia, fever, respiratory distress and sore throat.

James H. Brien

The history of this illness began 3 days before admission with upper respiratory infection symptoms with cough, shortness of breath, a sore throat and low-grade fever. On the day of admission, he was taken to a community hospital ED where pneumonia (Figure 1) and presumed strep throat was diagnosed, based on petechiae in his throat; he was given an injection of penicillin G benzathine and penicillin G procaine (Bicillin, Pfizer) before transfer.

He was treated with supplemental oxygen and bronchodilator therapy and empiric antibiotics (ceftriaxone). Soon after admission, his mouth lesions worsened, and by day 6 (day 9 of illness) had developed into severe mucositis of the mouth and lips, as well as conjunctivitis and urethritis.

His medical history is positive only for mild, intermittent asthma. He has otherwise been healthy, on no medications, and his immunizations are up-to-date. His mother had a cold about a week before the onset of symptoms in the patient. She recovered without any problems, and there have been no other known sick contacts. Dad recently returned from a deployment to Afghanistan with his Army unit, but he is well.

Figure 1. On the day of admission, he was taken to a community hospital ED where pneumonia was diagnosed.

Source: Brien JH

Examination on hospital day 6 revealed an alert adolescent male with normal vital signs (admission vital signs revealed fever of 101.9°F, respiratory rate of 30, pulse rate of 110); badly cracked and bleeding lips (Figure 2); bilateral conjunctival inflammation (Figure 3); skin lesions only on his scrotum, consisting of erosive lesions that had been vesicular (Figure 4); and inflammation of his urethra (Figure 5). The rest of his exam was normal, including the rest of his skin. Although he had a mild cough, his breath sounds were clear; however, he was noted to have had rhonchi and wheezing on the admission exam.

Examination revealed badly cracked and bleeding lips (Figure 2); bilateral conjunctival inflammation (Figure 3).

Skin lesions only on his scrotum, consisting of erosive lesions that had been vesicular (Figure 4); and inflammation of his urethra (Figure 5).

Admission lab tests were unremarkable, including non-detectable Mycoplasma IgG and IgM antibodies, except for a positive respiratory virus panel for rhinovirus. However, 7 days later, the Mycoplasma IgG was mildly elevated and the IgM was strongly elevated.

What's Your Diagnosis?

A. Atypical Stevens-Johnson syndrome
B. Kawasaki syndrome
C. Erythema multiforme minor
D. Toxic epidermal necrolysis

Case Discussion

The answer is A, atypical Stevens-Johnson syndrome; a very uncommon condition, closely associated with Mycoplasma infections. It is characterized by severe oral mucositis, vaginal and urethral inflammation, conjunctivitis, cracked/bleeding lips, but minimal to no skin manifestations that characteristically accompanies typical Stevens-Johnson syndrome. One study by Ravin and colleagues reported three cases within 1 month; eerily similar to three cases we had during a 4-month period: 1) the patient presented; 2) a 14-year-old male (Figure 6 and 7); and 3) a 15-year-old female (Figure 8). Two of our patients had serologic evidence of acute M. pneumonia infection, and one only had a positive IgG, with no other infectious cause documented. The photos above are of a 14-year-old male with typical Stevens-Johnson syndrome.

Figures 6 and 7 are of a 14-year-old male with typical Stevens-Johnson syndrome..

Kawasaki syndrome can certainly have some of the same clinical features (conjunctival inflammation without discharge, red lips and inflamed mucous membranes). However, a polymorphous rash is characteristic with Kawasaki, but not at all with atypical Stevens-Johnson syndrome. Kawasaki patients are almost always considerably younger, with 80% being aged 5 years or younger.

Erythema multiforme minor is a term falling out of favor; being replaced by erythema multiforme. These lesions are often triggered by infections, especially herpes simplex, and consist mostly of round or oval-shaped, fixed erythematous lesions that start as maculopapular lesions that progress to form larger circular areas of epidermal injury, often with vesiculation and central color change due to necrosis, to form target lesions within days to weeks (Figure 9). The palms and soles can also be involved. The term erythema multiforme major is also going back to being Stevens-Johnson syndrome.

Figure 8. A 15-year-old female with typical Stevens-Johnson syndrome.

The lesions in Figure 9 consist mostly of fixed erythematous lesions, often with vesiculation and central color change due to necrosis, to form “target” lesions within days to weeks. Toxic epidermal necrolysis is the consequence of a deeper level of cutaneous injury (subepidermal), usually brought on as a result of a reaction to a medication (Figure 10).

Toxic epidermal necrolysis (TEN) is the consequence of a deeper level of cutaneous injury (subepidermal), usually brought on as a result of a reaction to a medication (Figure 10), requiring more intensive skin care, similar to a burn patient. A key clinical feature is that the blisters of TEN have thicker overlying skin, so they are not as fragile and will persist through more manipulation, whereas the depth of injury from the epidermolytic toxins of staphylococcal scalded skin syndrome will be more superficial, with more flaccid and fragile blisters.

References:

Ravin KA. Pediatrics. 2007;119:e1002-e1005.

For more information:

James H. Brien, DO, is with the department of infectious diseases at McLane Children's Hospital, Baylor Scott & White Health, Texas A&M College of Medicine in Temple, Texas. He is also a member of the Infectious Diseases in Children Editorial Board. Brien can be reached at: jhbrien@aol.com.
Disclosure: Brien reports no relevant financial disclosures.