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Febrile ulceronecrotic Mucha–Habermann disease following suspected hemorrhagic chickenpox infection in a 20-month-old boy

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Abstract

We present the youngest pediatric patient so far with febrile ulcerative Mucha–Haberman disease (FUMHD) after an admitting clinical picture of hemorrhagic varicella infection. With a time to diagnosis of 25 days, the 20-month-old boy responded to low dose cyclosporine and prednisolone given for 3 months and is free of disease after 4 years of follow up. We describe a polyclonal CD8+ T cell response with elevated pro-inflammatory cytokines and a fivefold upregulation of the high-affinity Fc receptor type I (CD64) on granulocytes. Early consideration of FUMHD in the differential diagnosis of a systemic inflammatory disease combined with a generalized necrotizing rash is important for early and adequate management of children with this rare and challenging disease.

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Conflict of interest

On behalf of all authors, the corresponding author states that there is no conflict of interest.

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Authors and Affiliations

  1. Department of Pediatrics, University Medicine Greifswald, Ferdinand-Sauerbruchstrasse 1, 17475, Greifswald, Germany

    H. N. Lode, P. Lauenstein & R. Bruns

  2. Institute of Pathology, University Medicine Greifswald, Friedrich-Loeffler-Str. 23e, 17475, Greifswald, Germany

    P. Döring & F. Dombrowski

  3. DKD HELIOS Klinik Wiesbaden, Aukammallee 33, 65191, Wiesbaden, Germany

    P. Lauenstein

  4. Department of Paediatric Dermatology, Children’s Hospital Wilhelmstift, Liliencronstraße 130, 22149, Hamburg, Germany

    P. Hoeger

Authors
  1. H. N. Lode
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  2. P. Döring
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  3. P. Lauenstein
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  4. P. Hoeger
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  5. F. Dombrowski
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  6. R. Bruns
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Correspondence to H. N. Lode.

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Lode, H.N., Döring, P., Lauenstein, P. et al. Febrile ulceronecrotic Mucha–Habermann disease following suspected hemorrhagic chickenpox infection in a 20-month-old boy. Infection 43, 583–588 (2015). https://doi.org/10.1007/s15010-015-0726-5

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  • DOI: https://doi.org/10.1007/s15010-015-0726-5

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