Infection

, Volume 43, Issue 5, pp 583–588 | Cite as

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

  • H. N. Lode
  • P. Döring
  • P. Lauenstein
  • P. Hoeger
  • F. Dombrowski
  • R. Bruns
Case Report
First Online:
Received:
Accepted:

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.

Keywords

Mean Fluorescence Intensity Systemic Inflammatory Disease Varicella Infection Brivudin Vesicular Rash 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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Notes

Conflict of interest

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

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Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  • H. N. Lode
    • 1
  • P. Döring
    • 2
  • P. Lauenstein
    • 1
    • 3
  • P. Hoeger
    • 4
  • F. Dombrowski
    • 2
  • R. Bruns
    • 1
  1. 1.Department of PediatricsUniversity Medicine GreifswaldGreifswaldGermany
  2. 2.Institute of PathologyUniversity Medicine GreifswaldGreifswaldGermany
  3. 3.DKD HELIOS Klinik WiesbadenWiesbadenGermany
  4. 4.Department of Paediatric DermatologyChildren’s Hospital WilhelmstiftHamburgGermany

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