[Clinical analysis of 29 patients with clinically amyopathic dermatomyositis].
OBJECTIVE
To explore the clinical features and prognosis of clinically amyopathic dermatomyositis (C-ADM).


METHODS
The clinical data, including skin lesion, muscle involvement, and lung disease, of 29 patients with C-ADM, 6 males and 23 females with a male/female ratio of 1:3.83, aged 44 +/- 8, were analyzed. Skin biopsy was taken in 5 patients and lung HRCT was done in all the patients.


RESULTS
The mean age at onset was (44 +/- 8) years. All patients presented with characteristic skin lesions such as Gottron's papulae (91.3%); heliotropic periorbital erythremia (75.9%); V-sign rash (37.9%); shawl-sign rash (24.1%); and periungual erythema and telangiectasias (20.7%). Gottron's papule was most commonly seen on the dorsal aspect of elbow (86.2%), proximal interphalangeal joints (58.6%), metacarpophalangeal joints (48.3%), patellae (23.8%), hip (20.7%), shoulder (13.8%), and ankle (9.5%). The cutaneous histopathologic pictures of these patients were all compatible with the skin lesions of dermatomyositis. Interstitial lung disease (ILD) was found by lung HRCT in 19 patients (65.5%). All patients received steroids combined with immunosuppressants, but rapidly progressive ILD happened to some of the patients and finally led to death due to respiratory failure in 5 of them.


CONCLUSIONS
C-ADM is most commonly seen in the middle-aged women. ILD is the commonest respiratory problem arising in C-ADM patients and can be fatal, therefore should be properly treated.
Zhang
Zhuo-li
ZL
Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College, Beijing 100730, China.
Lai
Ai-yun
AY
Xu
Dong
D
Chen
Yi-he
YH
Tang
Fu-lin
FL
D000328:Adult; D001706:Biopsy; D003882:Dermatomyositis / Q000150:complications / Q000188:drug therapy / Q000473:pathology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000175:diagnosis / Q000209:etiology; D008297:Male; D008875:Middle Aged; D012867:Skin / Q000187:drug effects / Q000473:pathology*