PMID- 24127870
OWN - NLM
STAT- MEDLINE
DCOM- 20150413
LR  - 20140303
IS  - 1346-8138 (Electronic)
IS  - 0385-2407 (Linking)
VI  - 40
IP  - 11
DP  - 2013 Nov
TI  - Four mild but refractory cases of pemphigus foliaceus successfully treated with 
      intravenous immunoglobulin.
PG  - 869-73
LID - 10.1111/1346-8138.12280 [doi]
AB  - Intravenous immunoglobulin (IVIG) is a potential second line of therapy for 
      pemphigus, with increasing evidence of its effectiveness and safety, although 
      oral corticosteroids remain the first treatment for pemphigus. IVIG is usually 
      applied in severe cases of pemphigus, particularly pemphigus vulgaris (PV). 
      Pemphigus foliaceus (PF) caused by immunoglobulin PF autoantibodies to desmoglein 
      1 (Dsg1) is usually milder than PV. However, PF cases are occasionally resistant 
      to corticosteroids and require long-term treatment to control the disease, 
      leading to various adverse effects. IVIG was used in patients with relatively 
      mild PF, who were resistant to therapies with corticosteroids and dapsone. We 
      assessed the disease severity by Pemphigus Disease Area Index (PDAI) and measured 
      anti-Dsg1 antibody indices by enzyme-linked immunosorbent assay, before and 4 
      months after IVIG. Four Japanese female PF patients (57.3 +/- 8.6 years) were 
      treated with a single cycle of IVIG (400 mg/kg per day for five consecutive days) 
      in combination with the previous therapies. Within 1-2 months of addition of 
      IVIG, all PF cases showed remarkable improvement of skin lesions, and PDAI also 
      markedly decreased. For 2 years after IVIG, no apparent exacerbation was 
      observed. Anti-Dsg1 antibody indices decreased in all cases during the 2 years. 
      IVIG could be a potential treatment for not only severe cases of PV but also mild 
      and refractory cases of PF. IVIG may trigger the shift from intractable condition 
      to remission via non-pathogenic anti-Dsg1 antibodies or some mechanisms excluding 
      anti-Dsg1 antibody.
CI  - (c) 2013 Japanese Dermatological Association.
FAU - Kawakami, Tamihiro
AU  - Kawakami T
AD  - Department of Dermatology, Kurume University School of Medicine, Kurume 
      University Institute of Cutaneous Cell Biology, Kurume, Fukuoka, Japan; 
      Department of Dermatology, St Marianna University School of Medicine, Kawasaki, 
      Kanagawa, Japan.
FAU - Koga, Hiroshi
AU  - Koga H
FAU - Saruta, Hiroshi
AU  - Saruta H
FAU - Ueda, Akihiro
AU  - Ueda A
FAU - Inoue, Yoshihiko
AU  - Inoue Y
FAU - Soma, Yoshinao
AU  - Soma Y
FAU - Ishii, Norito
AU  - Ishii N
FAU - Hashimoto, Takashi
AU  - Hashimoto T
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20131016
PL  - England
TA  - J Dermatol
JT  - The Journal of dermatology
JID - 7600545
RN  - 0 (Antibodies)
RN  - 0 (DSG1 protein, human)
RN  - 0 (Desmoglein 1)
RN  - 0 (Glucocorticoids)
RN  - 0 (Immunoglobulins, Intravenous)
RN  - 9PHQ9Y1OLM (Prednisolone)
SB  - IM
MH  - Aged
MH  - Antibodies/blood
MH  - Desmoglein 1/immunology
MH  - Enzyme-Linked Immunosorbent Assay
MH  - Female
MH  - Glucocorticoids/administration & dosage
MH  - Humans
MH  - Immunoglobulins, Intravenous/*therapeutic use
MH  - Middle Aged
MH  - Pemphigus/*drug therapy/immunology/pathology
MH  - Prednisolone/administration & dosage
MH  - Skin/pathology
OTO - NOTNLM
OT  - corticosteroid
OT  - dapsone
OT  - desmoglein
OT  - intravenous immunoglobulin
OT  - pemphigus foliaceus
EDAT- 2013/10/17 06:00
MHDA- 2015/04/14 06:00
CRDT- 2013/10/17 06:00
PHST- 2013/06/16 00:00 [received]
PHST- 2013/08/13 00:00 [accepted]
PHST- 2013/10/17 06:00 [entrez]
PHST- 2013/10/17 06:00 [pubmed]
PHST- 2015/04/14 06:00 [medline]
AID - 10.1111/1346-8138.12280 [doi]
PST - ppublish
SO  - J Dermatol. 2013 Nov;40(11):869-73. doi: 10.1111/1346-8138.12280. Epub 2013 Oct 
      16.