PMID- 15840118
OWN - NLM
STAT- MEDLINE
DCOM- 20050714
LR  - 20131121
IS  - 0007-0963 (Print)
IS  - 0007-0963 (Linking)
VI  - 152
IP  - 4
DP  - 2005 Apr
TI  - Transition of pityriasis lichenoides et varioliformis acuta to febrile 
      ulceronecrotic Mucha-Habermann disease is associated with elevated serum tumour 
      necrosis factor-alpha.
PG  - 794-9
AB  - Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) represents a fulminant and 
      potentially lethal variant of pityriasis lichenoides. Only 24 cases have been 
      described so far. We report a 9-year-old boy who initially presented with 
      classical pityriasis lichenoides et varioliformis acuta (PLEVA) following a mild 
      enteritis. Three weeks later, his skin lesions started to ulcerate progressively, 
      involving > 90% of his body surface, accompanied by high fever, normal C-reactive 
      protein, but highly elevated serum levels of tumour necrosis factor (TNF)-alpha. 
      Methotrexate 10 mg m(-2) weekly was required to halt disease progression, while 
      oral steroids (initial dose 2.8 mg kg(-1) daily) alone proved insufficient. 
      Sequential histology revealed progressively dense perivascular and intramural 
      lymphocytic inflammation as well as keratinocyte necrosis. Our case demonstrates 
      the clinical and histological continuum between 'classical' PLEVA and FUMHD and 
      points to the potentially pathogenic significance of TNF-alpha. We hypothesize 
      that in future cases, treatment with TNF-alpha antagonists might represent a 
      reasonable alternative to high-dose immunosuppressive therapy.
FAU - Tsianakas, A
AU  - Tsianakas A
AD  - Division of Paediatric Dermatology, Department of Dermatology, University of 
      Hamburg, Martinistr. 52, D-20246 Hamburg, Germany.
FAU - Hoeger, P H
AU  - Hoeger PH
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Review
PL  - England
TA  - Br J Dermatol
JT  - The British journal of dermatology
JID - 0004041
RN  - 0 (Dermatologic Agents)
RN  - 0 (Interleukin-2)
RN  - 0 (Tumor Necrosis Factor-alpha)
RN  - 207137-56-2 (Interleukin-4)
RN  - YL5FZ2Y5U1 (Methotrexate)
SB  - IM
MH  - Administration, Oral
MH  - Child
MH  - Dermatologic Agents/administration & dosage
MH  - Fever/pathology
MH  - Humans
MH  - Interleukin-2/blood
MH  - Interleukin-4/blood
MH  - Male
MH  - Methotrexate/administration & dosage
MH  - Necrosis
MH  - Pityriasis Lichenoides/blood/drug therapy/*pathology
MH  - Skin Ulcer/pathology
MH  - Treatment Outcome
MH  - Tumor Necrosis Factor-alpha/*analysis
RF  - 26
EDAT- 2005/04/21 09:00
MHDA- 2005/07/15 09:00
CRDT- 2005/04/21 09:00
PHST- 2005/04/21 09:00 [pubmed]
PHST- 2005/07/15 09:00 [medline]
PHST- 2005/04/21 09:00 [entrez]
AID - BJD6485 [pii]
AID - 10.1111/j.1365-2133.2005.06485.x [doi]
PST - ppublish
SO  - Br J Dermatol. 2005 Apr;152(4):794-9. doi: 10.1111/j.1365-2133.2005.06485.x.