Extended report
Pneumomediastinum in dermatomyositis: association with cutaneous
vasculopathy
Hajime Kono, Shigeko Inokuma, Hisanori Nakayama, Mie Suzuki
Department of
Allergy and Immunological Diseases, Tokyo Metropolitan Komagome
Hospital, 3-18-22 Honkomagome, Bunkyo-ku, Tokyo 113-8677, Japan
Correspondence to: Dr Inokuma Email: inokuma-k{at}komagomehospital.bunkyo.tokyo.jp
Accepted for publication 2 November 1999
OBJECTIVES
To study
the pathogenesis of pneumomediastinum in polymyositis/dermatomyositis
(PM/DM).
PATIENTS AND
METHODSThe clinical records of 48 patients with
PM/DM were reviewed, focusing mainly on the presence of
pneumomediastinum and cutaneous vasculopathy, and the chest
radiographic changes. A patient with pneumomediastinum with a
characteristic change in his bronchus is described in detail. Case
reports of pneumomediastinum in PM/DM in English publications are reviewed.
RESULTSAmong the 48 patients with PM/DM, pneumomediastinum was observed as a complication
in four patients with DM and none of the patients with PM. Three of the
four patients with pneumomediastinum, but only six of the 44 patients
without this complication, had associated cutaneous vasculopathy. There
was a significant association of pneumomediastinum with cutaneous
vasculopathy (p = 0.02) and younger age (p = 0.04), but not with the
prevalence of lung disease. A 30 year old man (patient 1) with DM, who
had interstitial pneumonitis and skin ulceration due to vasculopathy,
developed pneumomediastinum. Fibreoptic bronchoscopy showed white
plaques on the bronchial mucosa, which were confirmed by microscopic
examination as representing subepithelial necrosis. A literature review
showed 13 cases of DM but no patient with PM with pneumomediastinum.
CONCLUSIONSIn patient
1, bronchial necrosis due to vasculopathy was strongly suspected as
being responsible for the pneumomediastinum. The results suggest that
pneumomediastinum was associated not with interstitial pneumonitis but
with the complication of vasculopathy appearing as skin lesions in DM.
© 2000 by Annals of the Rheumatic Diseases
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