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A neonate presents with fever, rash around the mouth
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A 3-week-old male presents with a fever and a rash. The history of the chief complaint began the day before admission, when the infant was noted to have a rash about the mouth and fussiness. As the fussiness was thought to be related to reflux (Sandifer’s syndrome), it was treated with Zantac (ranitidine, Boehringer Ingelheim), and topical Bactroban (mupirocin, GlaxoSmithKline) was applied about the lips for presumed impetigo, with arrangements for follow-up the next day. However, the infant became febrile that night, and was taken to the ER, where he had a fever of 103°F.
He received a full sepsis work-up and was given a dose of ampicillin and gentamicin, and he was admitted to the general pediatric service. His past medical history was that of a healthy newborn after a normal, uncomplicated pregnancy, labor and delivery. All prenatal tests were negative. The infant had received his first hepatitis B vaccine prior to discharge from the nursery. He had been on no medications and had no sick contacts, animal exposure or insect bites.
He was noted to have bright red skin from head to foot, along with some areas of apparent blistering, as there was redundant skin over what appeared to be patches of denuded, underlying skin (Figures 1-3).
Images: Brien JH
Figure 3.
Examination revealed an alert and active infant with a temperature of 99.9°F, but fever was documented in the ER as noted. Also the infant’s pulse was 170 and respiratory rate was 48, with normal oxygen saturations on room air. Although he was alert, the infant was very irritable, especially when handled or picked up, even by his mother.
He was noted to have bright red skin from head to foot, along with some areas of apparent blistering, as there was redundant skin over what appeared to be patches of denuded, underlying skin (Figures 1-3). Additionally, he had nasal congestion with some mucopurulent discharge; however, all his other mucous membranes — including eyes, lips, mouth urethra and anus — were clear.
Lab tests included a normal complete blood count, urinalysis and complete metabolic profile. The metabolic profile was normal but the C-reactive protein was 4.8. Blood, urine and nasal cultures are pending, but the spinal tap was unsuccessful, yielding no cerebrospinal fluid.
Case Discussion
This is a classic case of Ritter disease (D), also known as staphylococcal scalded skin syndrome (SSSS) in a neonate, as defined by Baron Gottfried Ritter von Rittershain (1820-1883). Rittershain reported the clinical features of this condition in 296 infants in a Czechoslovakian orphanage, or foundling facility, in 1878; he called it exfoliative neonatal dermatitis. A few years later, Staphylococcus was identified by Sir Alexander Ogston, MD, in 1881, and then Hans Christian Gram, MD, developed a standardized staining technique in 1885.
Ernst Almquist, MD, isolated S. aureus from a patient with SSSS in 1891. However, it was Alan Lyell, FRCP, who made the association of skin exfoliation with S. aureus in 1967 while working on toxic epidermal necrolysis (TEN). This was confirmed by Marian Melish, MD, and colleagues in 1972. Melish, of the University of Hawaii and Kapiolani Children’s Hospital, remains one of the icons of Staphylococcus toxin discovery.
These small molecular weight proteins (epidermolytic Toxins A and B) in the circulation produce the effects of SSSS by damaging the desmosomal cadherins, high in the granular layer of the epidermis, and disrupting the intracellular bonds. This damage creates a space that is quickly filled with “blister fluid,” raising a very thin-walled blister that can range in size from a small vesicle to large bullae.
These lesions rupture very easily, leaving behind the denuded skin below. This can obviously be fairly severe in newborns from an infection standpoint, as well as a fluid management standpoint. In older children, it is simply referred to as SSSS, but in newborns (infants up to 1 month of age), it is historically known as Ritter disease.
The treatment is antistaphylococcal antimicrobial therapy, drain any abscess, and remove any infected foreign body and supportive care. The Staphylococcus is not likely to be recovered from the blood, but can usually be found in the nose, which was the case with this infant. The other cultures were negative, and CSF was never obtained, as repeat lumbar puncture was not attempted, which was my recommendation. The infant did well and grew methicillin-sensitive S. aureus from his nose culture, and had an uneventful recovery (Figure 4).
Kawasaki disease would be very unusual at this age. A significant difference is that Kawasaki disease does not result in blistering of the skin, and can be taken off the list at that point.
Lyell disease, as alluded to above, is synonymous with TEN; a severe, life-threatening, deep, subepidermal toxin-mediated injury to the skin, with results similar to a third degree burn. Therefore, these children should be treated in a burn unit or similar facility. Antibiotics have no role in the treatment of the underlying condition, but just as burn patients, these patients are at risk for secondary infections.
The infant did well and grew methicillin-sensitive S. aureus from his nose culture, and had an uneventful recovery (Figure 4).
Lyell became a very noteworthy figure in the dermatology world through his life’s work. He was born in colonial India, the child of a British Army officer, but his mother died of puerperal fever. He was therefore raised by nannies. He was educated at Pembroke College and Cambridge, after which he attended Medical School at St. Thomas Hospital in London. He became a medical officer in the British Army during World War II, being wounded at Normandy (actually by “friendly fire”), and leading to his discharge in 1944.
He then went for dermatology training in 1946, and ended up at the Glasgow Royal Infirmary, where this work was done. He observed that TEN was triggered by a drug, resulting in a deeper injury than SSSS. At the Glasgow Royal Infirmary, a new dermatology clinic was named the Alan Lyell Center before his death in 2007 at age 90 years.
Stevens-Johnson syndrome was first described by Albert Stevens, MD, (1884-1945) and Frank Johnson, MD, (1894-1934) together in the American Journal of Diseases of Children, in 1922. This is an inflammatory condition causing a maculopapular, vesicular rash that may be triggered by medications or infections with agents such as herpes, Mycoplasma, etc.
While there may be vesicles and even some small blisters, Stevens-Johnson syndrome is not associated with a scalded skin-type appearance with large bullous lesions. It also has inflamed mucous membranes as a diagnostic criterion. For a while, this term was synonymous with erythema multiforme major, but recent changes in terminology suggest that we go back to referring to these patients as having Stevens-Johnson syndrome, and erythema multiforme minor as simply e. multiforme. Dermatology definitions and terminology changes keep my head spinning.
*Additional note: SSSS and TEN were briefly mentioned (with pictures) last month in the case of a 22-month-old male referred from a community hospital ER for admission with a blistering rash.
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- 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 also is 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.