Document Detail

Febrile Ulceronecrotic Mucha-Habermann Disease in a 34-Month-Old Boy: A Case Report and Review of the Literature.
MedLine Citation:
PMID:  21906156     Owner:  NLM     Status:  Publisher    
  We report a case of febrile ulceronecrotic Mucha-Habermann disease (FUMHD) in a 34-month-old boy. Our patient had a history of biopsy-proven pityriasis lichenoides et varioliformis acuta (PLEVA) since age 2. At 34 months, his skin lesions rapidly progressed to ulceration and necrosis in the setting of high fever. Skin biopsy revealed an intense lichenoid infiltrate with parakeratosis at the edges of areas of epidermal necrosis consistent with FUMHD, which is the severe variant of PLEVA. Despite initial treatment with prednisolone, his disease progressed to involve more than 50% of his body surface area. In addition to corticosteroids, he was treated with intravenous immunoglobulin, dapsone, and acyclovir, without complete resolution of disease. Methotrexate successfully cleared his skin disease and systemic symptoms. This patient highlights the exceptional response of FUMHD to methotrexate. Based on our review of the literature, this also represents the youngest reported case of FUMHD.
Bridget S Perrin; Albert C Yan; James R Treat
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-9-9
Journal Detail:
Title:  Pediatric dermatology     Volume:  -     ISSN:  1525-1470     ISO Abbreviation:  -     Publication Date:  2011 Sep 
Date Detail:
Created Date:  2011-9-12     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8406799     Medline TA:  Pediatr Dermatol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
© 2011 Wiley Periodicals, Inc.
School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania Pediatric Dermatology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
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