PMID- 11531959
OWN - NLM
STAT- MEDLINE
DCOM- 20011011
LR  - 20190513
IS  - 0009-9104 (Print)
IS  - 1365-2249 (Electronic)
IS  - 0009-9104 (Linking)
VI  - 125
IP  - 3
DP  - 2001 Sep
TI  - Diagnostic features of pemphigus vulgaris in patients with pemphigus foliaceus: 
      detection of both autoantibodies, long-term follow-up and treatment responses.
PG  - 492-8
AB  - There are several studies that describe the simultaneous presence and conversion 
      of pemphigus foliaceus into pemphigus vulgaris and vice versa. We describe eight 
      patients with clinical, histological and immunopathological features of pemphigus 
      foliaceus, at the time of the initial diagnosis. After a mean period of 2.5 
      years, additional serological features of pemphigus vulgaris were observed. 
      During a long-term follow-up, systemic therapies, their durations and treatment 
      outcomes were recorded. These patients did not respond to conventional systemic 
      therapy and developed multiple side-effects from these drugs. Hence, they were 
      treated with intravenous immunoglobulin therapy (IVIg). Prior to the initiation 
      of IVIg therapy, different assays were performed to detect the presence of 
      autoantibodies, including indirect immunofluorescence (IIF), immunoblot assay 
      using bovine gingival lysate, and ELISA. Twenty-five healthy normal individuals, 
      12 patients with pemphigus vulgaris, and eight patients with pemphigus foliaceus 
      served as controls for comparison of serological studies. At the time of initial 
      diagnosis, the sera of all eight study patients also demonstrated binding on an 
      immunoblot assay to a 160-kDa protein (desmoglein 1) only. This is typically 
      observed in pemphigus foliaceus. Prior to staring IVIg therapy, binding was 
      observed to both the 160 kDa and 130 kDa (desmoglein 3) proteins on an immunoblot 
      assay which was characteristic of pemphigus vulgaris. The antidesmogleins, 1 and 
      3 autoantibodies, were predominantly of the IgG4 subclass in all eight patients 
      studied. IVIg therapy induced remission in four patients and control in four of 
      the eight patients. The total follow-up period ranged from 2.6 to 9.5 years (mean 
      5.3 years). It is difficult to determine the exact time at which these patients 
      with pemphigus foliaceus developed pemphigus vulgaris. It is possible that the 
      disease was nonresponsive to conventional immunosuppressive therapy owing to the 
      simultaneous presence of two autoantibodies.
FAU - Sami, N
AU  - Sami N
AD  - Department of Oral Medicine, Harvard School of Dental Medicine, Boston, MA 02115, 
      USA.
FAU - Bhol, K C
AU  - Bhol KC
FAU - Ahmed, A R
AU  - Ahmed AR
LA  - eng
PT  - Clinical Trial
PT  - Journal Article
PL  - England
TA  - Clin Exp Immunol
JT  - Clinical and experimental immunology
JID - 0057202
RN  - 0 (Autoantibodies)
RN  - 0 (Autoantigens)
RN  - 0 (Cadherins)
RN  - 0 (DSG3 protein, human)
RN  - 0 (Desmoglein 1)
RN  - 0 (Desmoglein 3)
RN  - 0 (Immunoglobulin G)
RN  - 0 (Immunoglobulins, Intravenous)
RN  - 0 (Immunosuppressive Agents)
SB  - IM
MH  - Adult
MH  - Aged
MH  - Autoantibodies/*blood
MH  - Autoantigens/immunology
MH  - Cadherins/immunology
MH  - Desmoglein 1
MH  - Desmoglein 3
MH  - Female
MH  - Follow-Up Studies
MH  - Humans
MH  - Immunoglobulin G/blood/classification
MH  - Immunoglobulins, Intravenous/*therapeutic use
MH  - Immunosuppressive Agents/therapeutic use
MH  - Immunotherapy
MH  - Male
MH  - Middle Aged
MH  - Pemphigus/classification/*diagnosis/*therapy
PMC - PMC1906144
EDAT- 2001/09/05 10:00
MHDA- 2001/10/12 10:01
PMCR- 2002/09/01
CRDT- 2001/09/05 10:00
PHST- 2001/09/05 10:00 [pubmed]
PHST- 2001/10/12 10:01 [medline]
PHST- 2001/09/05 10:00 [entrez]
PHST- 2002/09/01 00:00 [pmc-release]
AID - cei1637 [pii]
AID - 10.1046/j.1365-2249.2001.01637.x [doi]
PST - ppublish
SO  - Clin Exp Immunol. 2001 Sep;125(3):492-8. doi: 10.1046/j.1365-2249.2001.01637.x.