PMID- 32358980
OWN - NLM
STAT- MEDLINE
DCOM- 20210617
LR  - 20210617
IS  - 1365-4632 (Electronic)
IS  - 0011-9059 (Linking)
VI  - 59
IP  - 10
DP  - 2020 Oct
TI  - Scleromyxedema treatment: a systematic review and update.
PG  - 1191-1201
LID - 10.1111/ijd.14888 [doi]
AB  - IMPORTANCE: Scleromyxedema is a chronic disease with high morbidity and mortality 
      and no definitive therapeutic guidelines. OBJECTIVE: To review all available data 
      on the efficacy and the safety of the available treatments of scleromyxedema and 
      suggest a possible therapeutic approach. EVIDENCE REVIEW: We performed a 
      systematic literature review in Pubmed/Medline, Embase, and Cochrane 
      collaboration databases, searching for all articles since 1990 on the treatments 
      of scleromyxedema, with no limits on participant age, gender, or nationality. 
      FINDINGS: Ninety-seven studies were included in this systematic review, of which 
      one prospective, two retrospective, 70 case reports/case series, and 24 
      letters/correspondence/clinical image. Intravenous immunoglobulin (IVIG) was the 
      most used first-line therapy based on its efficacy and its generally 
      well-tolerated nature; most patients require continued treatment to remain in 
      remission. Thalidomide and systemic glucocorticoids were mostly considered as 
      second-line therapies and were given alone or in association with IVIG. Patients 
      with severe or refractory disease were treated with autologous bone marrow 
      transplantation, melphalan, or bortezomib with dexamethasone. CONCLUSIONS AND 
      RELEVANCE: Consideration of patient comorbidities, disease distribution, 
      clinician experience, and treatment accessibility is mandatory in every 
      therapeutic approach of scleromyxedema.
CI  - (c) 2020 the International Society of Dermatology.
FAU - Haber, Roger
AU  - Haber R
AD  - Department of Dermatology, Saint George Hospital University Medical Center, 
      Beirut, Lebanon - Faculty of Medicine, Balamand University, Beirut, Lebanon.
FAU - Bachour, Julien
AU  - Bachour J
AD  - Department of Dermatology, Saint George Hospital University Medical Center, 
      Beirut, Lebanon - Faculty of Medicine, Balamand University, Beirut, Lebanon.
FAU - El Gemayel, Maria
AU  - El Gemayel M
AD  - Department of Gastroenterology, Hotel Dieu de France University Hospital, Beirut, 
      Lebanon - Faculty of Medicine, Saint Joseph University, Beirut, Lebanon.
LA  - eng
PT  - Journal Article
PT  - Review
PT  - Systematic Review
DEP - 20200502
PL  - England
TA  - Int J Dermatol
JT  - International journal of dermatology
JID - 0243704
RN  - 4Z8R6ORS6L (Thalidomide)
RN  - 69G8BD63PP (Bortezomib)
SB  - IM
MH  - Bortezomib
MH  - Humans
MH  - Prospective Studies
MH  - Retrospective Studies
MH  - *Scleromyxedema/diagnosis/drug therapy
MH  - Thalidomide/therapeutic use
EDAT- 2020/05/03 06:00
MHDA- 2021/06/22 06:00
CRDT- 2020/05/03 06:00
PHST- 2019/04/08 00:00 [received]
PHST- 2019/10/16 00:00 [revised]
PHST- 2020/03/26 00:00 [accepted]
PHST- 2020/05/03 06:00 [pubmed]
PHST- 2021/06/22 06:00 [medline]
PHST- 2020/05/03 06:00 [entrez]
AID - 10.1111/ijd.14888 [doi]
PST - ppublish
SO  - Int J Dermatol. 2020 Oct;59(10):1191-1201. doi: 10.1111/ijd.14888. Epub 2020 May 
      2.