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Clinical and Serological Transition From Pemphigus Vulgaris to Pemphigus Foliaceus Demonstrated by Desmoglein ELISA System
Yukiko Tsuji, MD;
Toshimitsu Kawashima, MD, PhD;
Koichi Yokota, MD, PhD;
Yasuki Tateish, MD;
Yuki Tomita, MD;
Tetsuri Matsumura, MD, PhD;
Hiroshi Shimizu, MD, PhD
Arch Dermatol. 2002;138:95-96.
INTRODUCTION
Several cases in which the disease course has undergone a transition
from pemphigus vulgaris (PV) to pemphigus foliaceus (PF) or vice versa have
been reported in the literature. The disease transition in these cases was
determined by means of clinical and histopathological observations and immunoblot
analysis. To our knowledge, the case described herein represents the first
report to clearly demonstrate the serological transition from mucous PV to
mucocutaneous PV to PF using a desmoglein (Dsg) enzyme-linked immunosorbent
assay (ELISA) system.
REPORT OF A CASE
A 55-year-old Japanese man presented to our outpatient clinic in March
1997 with a 3-month history of intractable oral erosions (Figure 1A). Clinical, histopathological, and immunological findings
led to the diagnosis of PV. Using a Dsg ELISA system (Dsg1/3 ELISA kit; Medical
& Biological Laboratories Co Ltd, Nagona, Japan), only anti-Dsg3 autoantibodies
(index, 214.07) were detectable (Figure 2A); the oral lesions had been well controlled with a dexamethasone
gargle.
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Figure 1. A, Oral erosion of mucous pemphigus
vulgaris. B, Erosions of mucocutaneous pemphigus vulgaris on the upper chest
area. C, Flaccid bullae and macerated crusts of pemphigus foliaceus.
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Figure 2. Desmoglein (Dsg) enzyme-linked
immunosorbent (ELISA) index of mucous pemphigus vulgaris (A), mucocutaneous
pemphigus vulgaris (B), and pemphigus foliaceus (C).
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In March 1999, the patient presented with newly developed oral lesions
as well as bullae and erosions on the trunk and extremities (Figure 1B). Histopathological analysis and immunofluorescence studies
of his skin blisters showed findings that were the same as those observed
at his first presentation. He was diagnosed as having mucocutaneous PV. At
this time, ELISA of his serum samples demonstrated both anti-Dsg1 (index,
163.76) and anti-Dsg3 (index, 173.45) autoantibodies (cutoff index value,
Dsg1, 9.78; Dsg3, 9.79) (Figure 2B).
He was treated with a combination of 1000 mg of methylprednisolone sodium
succinate pulse therapy, plasmapheresis, and 8 mg/d of betamethasone. When
the dosage of betamethasone therapy was reduced to 3.5 mg/d after the PV lesions
had cleared, flaccid bullae and macerated crusts developed on his face and
trunk, while he was completely clear of oral lesions (Figure 1C). Clinical and pathological findings led to a diagnosis
of PF. At this point, his serum sample contained only anti-Dsg1 autoantibodies
(Figure 12C).
The dosage of betamethasone therapy was increased to 6 mg/d, and 150
mg of azathioprine was added to the regimen. The lesions were gradually epithelialized,
and no new blister formation was observed.
COMMENT
It has been clearly demonstrated that serum samples from patients with
the mucosal dominant type of PV contain only anti-Dsg3 autoantibodies, while
those of patients with mucocutaneous PV contain both anti-Dsg1 and anti-Dsg3
autoantibodies. Therefore, the anti-Dsg autoantibody profile defines the clinical
phenotype of pemphigus.1 In the present case,
the patient experienced a clinical transition from the mucous dominant type
of PV to mucocutaneous PV to PF. We performed Dsg ELISA 15 times dur ing the
course of the disease (Figure 2),
and the ELISA titers and pemphigus phenotype as well as disease activities
proved to be closely correlated.
The course of disease in most patients with PV begins with mucous lesions;
subsequently, cutaneous lesions develop.2 In
such cases, epitope spreading from Dsg3 to Dsg1 might occur, but the reason
for the reduction in anti-Dsg3 autoantibodies during the disease course, as
seen in our case, is still unknown.
Ding et al3 demonstrated that anti-Dsg1
autoantibodies in PV serum samples are pathogenic and that they induce typical
PF lesions in neonatal mice. In case of transition, some immunological mechanism
may cause the reduction of anti-Dsg3 autoantibodies during the mucocutaneous
PV stage, and then the remainder of anti-Dsg1 autoantibodies cause PF lesions.
AUTHOR INFORMATION
Accepted for publication May 23, 2001.
Corresponding author and reprints: Yukiko Tsuji, MD, Department of
Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, 060-8638
Japan (e-mail: yutsuji{at}med.hokudai.ac.jp).
From the Department of Dermatology, Hokkaido University Graduate School
of Medicine, Sapporo, Japan.
REFERENCES
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1. Amagai M, Tsunoda K, Zillikens D, Nagai T, Nishikawa T. The clinical phenotype of pemphigus is defined by the anti-desmoglein
autoantibody profile. J Am Acad Dermatol. 1999;40:167-170.
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2. Korman N. Pemphigus. J Am Acad Dermatol. 1988;18:1219-1238.
WEB OF SCIENCE
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3. Ding X, Diaz LA, Fairley JA, Giudice GJ, Liu Z. The anti–desmoglein 1 autoantibodies in pemphigus vulgaris sera
are pathogenic. J Invest Dermatol. 1999;112:739-743.
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