title: Chronic pulmonary complications associated with toxic epidermal necrolysis: report of a severe case with anti-Ro/SS-A and a review of the published work.
abstract: Patients with toxic epidermal necrolysis (TEN) have been known to have various complications. Though pulmonary complications are often observed, they usually show an acute form; however, chronic complications are quite rare and little is known about either their incidences or clinical manifestations. We herein report a 33-year-old man who presented with chronic pulmonary complications after a recovery from TEN. At the onset of TEN, he had severe respiratory failure and artificial ventilation was instituted. Despite being extubated successfully, respiratory failure reappeared 1 month later. A diagnosis of chronic bronchitis with severe obstructive ventilatory impairment and bronchiectasis was made and he was treated with steroids, bronchodilators and antibiotics, however, he died 1.5 years after the onset of TEN. There have been 13 reported cases of chronic pulmonary complications with TEN or Stevens-Johnson syndrome (SJS) in the English published work. Such cases are usually classified into chronic bronchitis/bronchiolitis with obstructive change (including bronchiolitis obliterans and bronchiolitis obliterans organizing pneumonia), respiratory tract obstruction and bronchiectasis. Approximately 40% of all such patients die while the surviving continue to suffer from these complications because no curative therapy yet exists. As a result, the prognosis seems to be poor. The relationship between TEN and these chronic pulmonary complications remains to be elucidated. Interestingly, our patient was asymptomatically anti-Ro/SS-A positive at the onset of TEN. In addition, eccrine gland involvement and an extremely high level of serum salivary amylase were observed at the onset of TEN, furthermore, Sjögren-like symptoms occurred after recovery from TEN. These findings suggested that the Sjögren-like autoimmune abnormalities induced by anti-Ro/SS-A correlated with the development of chronic pulmonary complications in our patient.
authors.affiliation: Department of Dermatology, Graduate School of Medicine, Chiba University, Japan. ozmail@faculty.chiba-u.jp
authors.forename: Noriaki
authors.initials: N
authors.lastname: Kamada
authors.forename: Kyoko
authors.initials: K
authors.lastname: Kinoshita
authors.forename: Yaei
authors.initials: Y
authors.lastname: Togawa
authors.forename: Takashi
authors.initials: T
authors.lastname: Kobayashi
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Matsubara
authors.forename: Masakazu
authors.initials: M
authors.lastname: Kohno
authors.forename: Hidetoshi
authors.initials: H
authors.lastname: Igari
authors.forename: Takayuki
authors.initials: T
authors.lastname: Kuriyama
authors.forename: Masataka
authors.initials: M
authors.lastname: Nakamura
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Hirasawa
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Shinkai
mesh_terms: D000328:Adult; D000974:Antibodies, Antinuclear / Q000502:physiology*; D002908:Chronic Disease; D006801:Humans; D008171:Lung Diseases / Q000209:etiology*; D008297:Male; D013262:Stevens-Johnson Syndrome / Q000150:complications*
chemical_list: D000974:Antibodies, Antinuclear; C035356:SS-A antibodies