A 37-year-old male welder, married to two children, with DM diagnosed at 35 years of age in another hospital, due to proximal muscle weakness, elevated creatine kinase (CK) skin, electromyography and biopsy.
No history of smoking, alcohol, drugs, or other diseases.
She received corticoids and azathioprine, which was suspended in the context of septic arthritis of the left knee.
She subsequently received methotrexate, which was suspended due to anemia.
Oral cyclophosphamide was started and, due to a change of address, controls were initiated in the rheumatology service of Padre Hurtado Hospital after two years of disease.
The admission showed severe muscle weakness, body mass index of 16, with muscle weakness predominantly proximal, M2 in lower limbs and M3 in upper limbs, which seemed to be determined more by disease than by activity.
In addition, a necrotic plaque was evidenced that compromised the distal area of the second finger of the left foot, with long-standing purulent exudate, which required numerous nursing dressings, without being able to heal.
No associated Raynaud.
The patient was hospitalized due to suspicion of infectious involvement of the ear.
Laboratory tests revealed hematocrit 3, hemoglobin 12.4 mg/dL, leukocytes 2,600, platelets 243,000, CRP 6, creatinine 0.36, BUN 14, normal CK.
The radiographs showed an osteomyelitis so we proceeded to the amputation of the compromised orthoses.
In addition, computed axial tomography (CAT) of the chest, abdomen and pelvis was performed in the context of the study of myopathy, which revealed an extensive Nm associated with incipient subcutaneous soft tissue emphysema, mild subpleural interstitial lung disease.
Endoscopy was performed and the patient was normal.
After cyclophosphamide was discontinued, leucopenia.
Muscle biopsy revealed irregular muscle fibers with marked signs of perimysial inflammatory cells and signs.
Antinuclear antibodies, anti-DNA, anti-neutrophil cytoplasmic antibodies, anti-cardiolipin antibodies, B2 glycoprotein 1 and extractable nuclear antigens were absent, as well as the lupus anticoagulant.
After discharge, treatment was initiated with hydroxychloroquine, azathioprine, intestinal loop, sildenafil and aspirin, with good initial response but a new skin lesion, similar to the previous one, in the right 5.
An attempt was made to treat the patient medically but with bone involvement so amputation of the distal phalanx was performed.
The control chest CT showed a decrease in Nm.
After this the patient had a good clinical evolution, BMI 24, without muscle weakness, normal CK, or respiratory compromise.
Only superficial, non-necrotic skin lesions that have appeared in other areas persist.
