A 49-year-old woman was admitted in February 1987, with a six-month history of
joint pain, maculopapular and erythematous
rash, proximal
muscle weakness and a two-month history of skin ulceration and
dyspnea on exertion. Physical examination showed Gottron's papules on her fingers and a faint heliotrope
rash. Biopsy of erythematous skin lesions on the shoulder and the back of the hand revealed perivascular inflammatory cell infiltration and tiny ulcerative lesions of the cutaneous tissue. Biopsy of the right quadriceps muscle showed type II fiber
atrophy and slight perivascular lymphocytic infiltrate, whereas serum CPK level was within normal range. Chest
X-ray film showed granular infiltrates in both lower lung fields. Based on the current findings the case was diagnosed as
dermatomyositis associated with
interstitial pneumonia. The administration of
prednisolone, 30 mg/day resulted in the improvement of the skin lesions and
muscle weakness, while the intensity of lung infiltrates was little affected. Three months after
steroid therapy, the patient was readmitted because of increasing
dyspnea on exertion and multiple
skin ulcers. Chest X-ray revealed a small amount of gas in the mediastinum, in addition to slight deterioration of
interstitial lung disease. In spite of various treatments for
pneumomediastinum, including
bed rest, administration of
analgesics and
oxygen inhalation, it developed rapidly, complicated severe
subcutaneous emphysema and right-sided
pneumothorax. Although high-dose
prednisolone therapy and mediastinal drainage were performed, the
pneumomediastinum was not resolved and she died from
respiratory failure. At autopsy, predominant histological features of the lungs were
acute interstitial pneumonia with hyaline membrane and edematous granulation formation in the alveoli.(ABSTRACT TRUNCATED AT 250 WORDS)