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Images in paediatrics
A case of recurrent toxin-mediated perineal erythema
  1. Rachida El Bouch1,3,
  2. Pauline Reubsaet2,
  3. Klaas Koop3,
  4. Jurgen Jansen3
  1. 1 Sint Lucas Andreas Hospital, Amsterdam, The Netherlands
  2. 2 University Medical Center Utrecht, Utrecht, The Netherlands
  3. 3 Meander Medical Center, Amersfoort, The Netherlands
  1. Correspondence to Klaas Koop, Meander Medical Center, Postbus 1502, Amersfoort 3800 BM, The Netherlands; klaaskoop{at}

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A 2-year-old girl presented with recurrent episodes of pharyngitis and a perineal rash. During the first episode, a course of antibiotics resulted in a quick resolution of symptoms. Yet a few days later, her symptoms had recurred and she was referred to our clinic. She was febrile, her lips were red and fissured (figure 1A), the tonsils were enlarged and red, she had a pronounced perineal rash (figure 1B) and, after a few days, desquamation of fingertips. Cultures of the throat showed an exotoxin C-producing group A streptococcus. She recovered with another course of antibiotics. A few months later, she had another episode of sore throat accompanied by perineal rash.

Figure 1

The patient presented with red, fissured lips (A) and a sharply demarcated rash of the perineal area (B). Desquamation of the fingertips (C) occurred a few days after the initial presentation.

The recurrent episodes of pharyngitis and perineal erythema associated with a toxin-producing group A streptococcus are compatible with ‘recurrent toxin-mediated perineal erythema’ (RTPE).1–3 RTPE is thought to result from bacterial toxins that act as superantigens.4 During the infection, a distinct perineal rash develops, along with a scarlet fever-like presentation. The patient is generally well, with no or only mild fever. About a week after the onset of the infection, desquamation of hands and feet is seen.1

The differential diagnoses of RTPE includes local infection, other toxin-mediated diseases and vasculitides. There is a considerable overlap with the clinical picture of Kawasaki's disease, and in the presence of prolonged fever, (incomplete) Kawasaki's disease will be difficult to rule out.5

In RTPE, antibiotics result in a quick resolution of symptoms. If symptoms recur frequently, tonsillectomy should be considered.


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  • Contributors All authors were involved in the clinical management of the patient. JJ had end-responsibility for the management of the patient. REB and PR wrote the initial draft of the manuscript, all other authors contributed to the final version of the manuscript. All authors have approved the final version of the manuscript.

  • Competing interests None.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; internally peer reviewed.

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