Cutaneous deep necrosis with dermatomyositis: correlation with interstitial pneumonia.

摘要

Interstitial pneumonia (IP) is a major complication of dermatomyositis (DM) and it often results in poor prognosis. In this report, we focus on cutaneous necrosis as a severe complication of DM and discuss the relationship between cutaneous necrosis and IP in DM. A 63-year-old Japanese woman presented with a 4-month history of general fatigue and a rash on the skin of her face and hands. Physical examination revealed erythema and swelling of the eyelids, compatible with a heliotropic rash, and keratotic erythematous patches on the knuckles, arms, elbows, and knees compatible with Gottron's sign. Despite the lack of muscular weakness, electromyography indicated myogenic abnormalities and laboratory data demonstrated a slight increase in serum aldolase level (6.0 lU/dL; normal range, 1.7-5.7 lU/dL) with normal serum levels of creatine phosphokinase. -The patient was negative for anti-nuclear antibody, anti-DNA antibody, anti-Jo-1 antibody, anti-phospholipid antibodies, and lupus anticoagulant. Chest X-ray and CT scan showed diffuse interstitial pneumonia and pulmonary function tests showed a restrictive pattern. The serum level of KL-6 was increased (1347 U/mL; normal range, 0-500 U/mL). Based on these findings, a diagnosis of DM with IP was made and pulse therapy with intravenous methy-lprednisolone (total of 3 g) followed by oral predniso-lone at a dose of 45 mg daily was started. Steroid therapy produced a partial response in the IP and the serum aldolase level decreased to within the normal range, which allowed gradual tapering of the steroid dose. An extensive systemic evaluation, which included X-ray examination, abdominal echogram, chest and abdominal CT scans, gastrofibroscopy, scanning, mammography, gynecological examination, and examination of serology markers, revealed no associated malignancy. One month after the initial treatment, the patient developed multiple cutaneous necrotic lesions with a purplish reticular livedo pattern on the dorsal side of the finger joints (figure 1A), elbows, andknees (figure IB), resulting in deep ulcerations after removal of the necrotic tissue. Skin biopsy from the vicinity of the finger lesions indicated perivascular lymphocytic infiltration and thickening of the vascular walls resulting in obstruction or narrowing of the vascular lumen in the dermis, but necrotizing vasculitis was not confirmed. The ulcers re-epithelialized gradually due to continuous local treatment, and complete healing took over 6 months.