title: Febrile ulceronecrotic Mucha-Habermann disease following suspected hemorrhagic chickenpox infection in a 20-month-old boy.
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.
authors: Department of Pediatrics, University Medicine Greifswald, Ferdinand-Sauerbruchstrasse 1, 17475, Greifswald, Germany. lode@uni-greifswald.de.
authors: H N
authors: HN
authors: Lode
authors: Institute of Pathology, University Medicine Greifswald, Friedrich-Loeffler-Str. 23e, 17475, Greifswald, Germany.
authors: P
authors: P
authors: Döring
authors: Department of Pediatrics, University Medicine Greifswald, Ferdinand-Sauerbruchstrasse 1, 17475, Greifswald, Germany.|DKD HELIOS Klinik Wiesbaden, Aukammallee 33, 65191, Wiesbaden, Germany.
authors: P
authors: P
authors: Lauenstein
authors: Department of Paediatric Dermatology, Children's Hospital Wilhelmstift, Liliencronstraße 130, 22149, Hamburg, Germany.
authors: P
authors: P
authors: Hoeger
authors: Institute of Pathology, University Medicine Greifswald, Friedrich-Loeffler-Str. 23e, 17475, Greifswald, Germany.
authors: F
authors: F
authors: Dombrowski
authors: Department of Pediatrics, University Medicine Greifswald, Ferdinand-Sauerbruchstrasse 1, 17475, Greifswald, Germany.
authors: R
authors: R
authors: Bruns
mesh_terms: D000900:Anti-Bacterial Agents / Q000008:administration & dosage; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage; D018414:CD8-Positive T-Lymphocytes / Q000276:immunology; D002644:Chickenpox / Q000150:complications*; D016572:Cyclosporine / Q000008:administration & dosage; D006098:Granulocytes / Q000737:chemistry / Q000276:immunology; D006561:Herpes Simplex / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology*; D006801:Humans; D007223:Infant; D008297:Male; D017514:Pityriasis Lichenoides / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology*; D011239:Prednisolone / Q000008:administration & dosage; D017452:Receptors, IgG / Q000032:analysis; D016896:Treatment Outcome
chemical_list: D000900:Anti-Bacterial Agents; D000893:Anti-Inflammatory Agents; C489017:FCGR1A protein, human; D017452:Receptors, IgG; D016572:Cyclosporine; D011239:Prednisolone
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