Epidermolysis bullosa acquisita

Clinical response to plasma exchange therapy and circulating anti-basement membrane zone antibody titer

Masutaka Furue, Mitsuru Iwata, Hong Il Yoon, Yasuo Kubota, Hitoshi Ohto, Makoto Kawashima, Tetsuya Tsuchida, Kuniaki Oohara, Kunihiko Tamaki, Atsushi Kukita

Research output: Contribution to journalArticle

36 Citations (Scopus)

Abstract

Epidermolysis bullosa acquisita has been recognized as a rare autoimmune mechanobullous disorder since the detection of immunoglobulin and complement deposits along the basement membrane zone. A circulating anti-basement membrane zone antibody has also been detected in some cases. We are reporting a case of epidermolysis bullosa acquisita in which clinical symptoms were well correlated with the circulating anti-basement membrane zone antibody titers. Although the patient initially responded very well to corticosteroid therapy, remission could not be maintained without increasing the dosage. Other therapies, including azathioprine, dapsone, vitamin E, and gold sodium thiomalate, produced no beneficial effects. Although a high dose of oral corticosteroid and cyclophosphamide decreased the antibody titer and blister formation, this therapy had to be terminated because of side effects. Plasma exchange therapy in combination with corticosteroid and low-dose cyclophosphamide resulted in a marked decrease of the anti-basement membrane zone antibody titer and clinical improvement. Thus plasma exchange therapy may be a useful adjunct to conventional treatments for patients with epidermolysis bullosa acquisita.

Original languageEnglish
Pages (from-to)873-878
Number of pages6
JournalJournal of the American Academy of Dermatology
Volume14
Issue number5
DOIs
Publication statusPublished - Jan 1 1986
Externally publishedYes

Fingerprint

Epidermolysis Bullosa Acquisita
Plasma Exchange
Basement Membrane
Antibodies
Adrenal Cortex Hormones
Cyclophosphamide
Therapeutics
Gold Sodium Thiomalate
Dapsone
Azathioprine
Blister
Vitamin E
Immunoglobulins

All Science Journal Classification (ASJC) codes

  • Dermatology

Cite this

Epidermolysis bullosa acquisita : Clinical response to plasma exchange therapy and circulating anti-basement membrane zone antibody titer. / Furue, Masutaka; Iwata, Mitsuru; Yoon, Hong Il; Kubota, Yasuo; Ohto, Hitoshi; Kawashima, Makoto; Tsuchida, Tetsuya; Oohara, Kuniaki; Tamaki, Kunihiko; Kukita, Atsushi.

In: Journal of the American Academy of Dermatology, Vol. 14, No. 5, 01.01.1986, p. 873-878.

Research output: Contribution to journalArticle

Furue, Masutaka ; Iwata, Mitsuru ; Yoon, Hong Il ; Kubota, Yasuo ; Ohto, Hitoshi ; Kawashima, Makoto ; Tsuchida, Tetsuya ; Oohara, Kuniaki ; Tamaki, Kunihiko ; Kukita, Atsushi. / Epidermolysis bullosa acquisita : Clinical response to plasma exchange therapy and circulating anti-basement membrane zone antibody titer. In: Journal of the American Academy of Dermatology. 1986 ; Vol. 14, No. 5. pp. 873-878.
@article{ae9a8c551f6d4328837bc6503d148e41,
title = "Epidermolysis bullosa acquisita: Clinical response to plasma exchange therapy and circulating anti-basement membrane zone antibody titer",
abstract = "Epidermolysis bullosa acquisita has been recognized as a rare autoimmune mechanobullous disorder since the detection of immunoglobulin and complement deposits along the basement membrane zone. A circulating anti-basement membrane zone antibody has also been detected in some cases. We are reporting a case of epidermolysis bullosa acquisita in which clinical symptoms were well correlated with the circulating anti-basement membrane zone antibody titers. Although the patient initially responded very well to corticosteroid therapy, remission could not be maintained without increasing the dosage. Other therapies, including azathioprine, dapsone, vitamin E, and gold sodium thiomalate, produced no beneficial effects. Although a high dose of oral corticosteroid and cyclophosphamide decreased the antibody titer and blister formation, this therapy had to be terminated because of side effects. Plasma exchange therapy in combination with corticosteroid and low-dose cyclophosphamide resulted in a marked decrease of the anti-basement membrane zone antibody titer and clinical improvement. Thus plasma exchange therapy may be a useful adjunct to conventional treatments for patients with epidermolysis bullosa acquisita.",
author = "Masutaka Furue and Mitsuru Iwata and Yoon, {Hong Il} and Yasuo Kubota and Hitoshi Ohto and Makoto Kawashima and Tetsuya Tsuchida and Kuniaki Oohara and Kunihiko Tamaki and Atsushi Kukita",
year = "1986",
month = "1",
day = "1",
doi = "10.1016/S0190-9622(86)70103-5",
language = "English",
volume = "14",
pages = "873--878",
journal = "Journal of the American Academy of Dermatology",
issn = "0190-9622",
publisher = "Mosby Inc.",
number = "5",

}

TY - JOUR

T1 - Epidermolysis bullosa acquisita

T2 - Clinical response to plasma exchange therapy and circulating anti-basement membrane zone antibody titer

AU - Furue, Masutaka

AU - Iwata, Mitsuru

AU - Yoon, Hong Il

AU - Kubota, Yasuo

AU - Ohto, Hitoshi

AU - Kawashima, Makoto

AU - Tsuchida, Tetsuya

AU - Oohara, Kuniaki

AU - Tamaki, Kunihiko

AU - Kukita, Atsushi

PY - 1986/1/1

Y1 - 1986/1/1

N2 - Epidermolysis bullosa acquisita has been recognized as a rare autoimmune mechanobullous disorder since the detection of immunoglobulin and complement deposits along the basement membrane zone. A circulating anti-basement membrane zone antibody has also been detected in some cases. We are reporting a case of epidermolysis bullosa acquisita in which clinical symptoms were well correlated with the circulating anti-basement membrane zone antibody titers. Although the patient initially responded very well to corticosteroid therapy, remission could not be maintained without increasing the dosage. Other therapies, including azathioprine, dapsone, vitamin E, and gold sodium thiomalate, produced no beneficial effects. Although a high dose of oral corticosteroid and cyclophosphamide decreased the antibody titer and blister formation, this therapy had to be terminated because of side effects. Plasma exchange therapy in combination with corticosteroid and low-dose cyclophosphamide resulted in a marked decrease of the anti-basement membrane zone antibody titer and clinical improvement. Thus plasma exchange therapy may be a useful adjunct to conventional treatments for patients with epidermolysis bullosa acquisita.

AB - Epidermolysis bullosa acquisita has been recognized as a rare autoimmune mechanobullous disorder since the detection of immunoglobulin and complement deposits along the basement membrane zone. A circulating anti-basement membrane zone antibody has also been detected in some cases. We are reporting a case of epidermolysis bullosa acquisita in which clinical symptoms were well correlated with the circulating anti-basement membrane zone antibody titers. Although the patient initially responded very well to corticosteroid therapy, remission could not be maintained without increasing the dosage. Other therapies, including azathioprine, dapsone, vitamin E, and gold sodium thiomalate, produced no beneficial effects. Although a high dose of oral corticosteroid and cyclophosphamide decreased the antibody titer and blister formation, this therapy had to be terminated because of side effects. Plasma exchange therapy in combination with corticosteroid and low-dose cyclophosphamide resulted in a marked decrease of the anti-basement membrane zone antibody titer and clinical improvement. Thus plasma exchange therapy may be a useful adjunct to conventional treatments for patients with epidermolysis bullosa acquisita.

UR - http://www.scopus.com/inward/record.url?scp=0022450147&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0022450147&partnerID=8YFLogxK

U2 - 10.1016/S0190-9622(86)70103-5

DO - 10.1016/S0190-9622(86)70103-5

M3 - Article

VL - 14

SP - 873

EP - 878

JO - Journal of the American Academy of Dermatology

JF - Journal of the American Academy of Dermatology

SN - 0190-9622

IS - 5

ER -