Published online August 1, 2007
PEDIATRICS Vol. 120 No. 2 August 2007, pp. 451-452 (doi:10.1542/peds.2007-1072)

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LETTER TO THE EDITOR

"Atypical" Stevens-Johnson Syndrome?

Peter C. Schalock, MD
Department of Dermatology
Massachusetts General Hospital/Harvard Medical School
Boston, MA 02114

James G. H. Dinulos, MD
Department of Pediatrics (Dermatology)
Dartmouth-Hitchcock Medical Center/Dartmouth Medical School
Lebanon, NH 03756

To the Editor.—

We read with interest the excellent case series by Ravin et al,¹ who examined one of the many manifestations of Mycoplasma pneumoniae infection. The 3 cases were excellent examples of isolated mucositis occurring in association with M pneumoniae infection. In their article, and a recent similar report of isolated mucositis,² the label of "atypical Stevens-Johnson syndrome" was applied to patients who developed the typical mucous membrane changes seen in Stevens-Johnson syndrome (SJS) but lacked cutaneous lesions. According to any of the classification systems of SJS, beginning with the original Stevens and Johnson³ description and up to the recent Bastuji-Garin et al⁴ criteria, diagnosis of SJS requires skin involvement. Clarity of terminology is important not only for communication between providers but also for setting therapeutic expectations. The addition of "atypical" in front of a known syndrome or disease makes complete sense when the recognition of atypical features and early diagnosis makes sense for early treatment (eg, adding "atypical" to Kawasaki disease for children with an incomplete clinical presentation). Alerting clinicians to atypical presentations should lead to early diagnosis and treatment. Unlike atypical Kawasaki disease, children with Mycoplasma-induced mucositis (or atypical SJS, as some refer to this condition) do not progress to having skin-barrier compromise and seem to have a better prognosis than patients with SJS.⁵

We believe that mucous membrane–limited disease resulting from M pneumoniae infection, considered within the spectrum of disease that encompasses bullous erythema multiforme and SJS, should not be referred to as atypical SJS. All 3 conditions can be caused by M pneumoniae infections, but bullous erythema multiforme and isolated mucositis have much better prognoses.⁵ Nonetheless, children with isolated mucositis must be monitored closely for skin signs that indicate progression to frank SJS. The widespread blistering seen in SJS may not be apparent for several days after mucous membrane changes. Ravin et al¹ stated that making the diagnosis of SJS without skin findings was "clinically challenging." We could not agree more completely and suggest that lack of fit for SJS may be solved by considering isolated mucositis as a separate, yet associated, diagnosis for this distinct mucous membrane manifestation of M pneumoniae infection.

REFERENCES

1. Ravin KA, Rappaport LD, Zuckerbraun NS, Wadowsky RM, Wald ER, Michaels MM. Mycoplasma pneumoniae and atypical Stevens-Johnson syndrome: a case series. Pediatrics. 2007;119(4) . Available at: www.pediatrics.org/cgi/content/full/119/4/e1002
2. Zipitis CS, Thalange N. Intravenous immunoglobulins for the management of Stevens-Johnson syndrome with minimal skin manifestations. Eur J Pediatr. 2006;166 :585 –588[Web of Science][Medline]
3. Stevens AM, Johnson FC. A new eruptive fever associated with stomatitis and ophthalmia. Am J Dis Child. 1922;24 :526 –533[Abstract/Free Full Text]
4. Bastuji-Garin S, Rzany B, Stern RS, Shear NH, Naldi L, Roujeau JC. Clinical classification of cases of toxic epidermal necrolysis, Steven-Johnson syndrome, and erythema multiforme. Arch Dermatol. 1993;129 :92 –96[Abstract/Free Full Text]
5. Schalock PC, Dinulos JG, Pace N, Schwarzenberger K, Wenger JK. Erythema multiforme due to Mycoplasma pneumoniae infection in two children. Pediatr Dermatol. 2006;23 :546 –555[CrossRef][Web of Science][Medline]

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PEDIATRICS (ISSN 1098-4275). ©2007 by the American Academy of Pediatrics

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This Article Extract Full Text (PDF) Submit a response Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Services E-mail this article to a friend Similar articles in this journal Similar articles in Web of Science Similar articles in PubMed Alert me to new issues of the journal Add to My File Cabinet Download to citation manager Request Permissions Citing Articles Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Schalock, P. C. Articles by Dinulos, J. G. H. Search for Related Content PubMed PubMed Citation Articles by Schalock, P. C. Articles by Dinulos, J. G. H. Related Collections Infectious Disease & Immunity Related AAP Red Book topics: Mycoplasma pneumoniae and Other... Social Bookmarking                         What's this?