Calcinosis cutis and intestinal pseudoobstruction in a patient with adult onset Still's disease associated with recurrent relapses of disordered coagulopathy.
Adult onset Still's disease (AOSD) is a systemic inflammatory disorder of unknown origin, characterized by a typical spiking fever, evanescent salmon-colored rash, polyarthralgia, and myalgia. Calcinosis cutis and gastrointestinal involvement have rarely been noted in AOSD. We herein describe a 54-year-old woman who demonstrated repeated disseminated intravascular coagulation (DIC), and adult respiratory distress syndrome (ARDS), associated with AOSD. The patient also revealed a remarkable degree of digital calcinosis cutis and intestinal pseudoobstruction. A connective tissue disease, such as systemic sclerosis, might have been the underlying factor in the latter two symptoms.
First Department of Internal Medicine, National Defense Medical College, Tokorozawa, Saitama.
T
T
Shinohara
T
T
Hidaka
Y
Y
Matsuki
K
K
Suzuki
F
F
Ohsuzu
D000925:Anticoagulants / Q000627:therapeutic use; D002114:Calcinosis / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D003937:Diagnosis, Differential; D004211:Disseminated Intravascular Coagulation / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D016670:Gabexate / Q000627:therapeutic use; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007418:Intestinal Pseudo-Obstruction / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D008875:Middle Aged; D010956:Plasmapheresis; D012008:Recurrence; D012121:Respiration, Artificial; D012128:Respiratory Distress Syndrome / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D012871:Skin Diseases / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D016706:Still's Disease, Adult-Onset / Q000150:complications* / Q000175:diagnosis / Q000628:therapy
D000925:Anticoagulants; D005938:Glucocorticoids; D016670:Gabexate