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Fatal vascular occlusion in juvenile dermatomyositis
  1. T TAKEDA,
  3. S JODO,
  4. T KOIKE
  1. Department of Medicine II
  2. Department of Pathology, Hokkaido University School of Medicine, Sapporo, Japan
  1. Dr T Takeda, Department of Medicine II, Hokkaido University School of Medicine, Kita 15-jo, Nishi 7-chome, Kita-ku, Sapporo 060, Japan.

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Juvenile dermatomyositis (JDMS) is a relatively rare disease characterised by vasculopathy.1-4 Involvement of the gastrointestinal tract may occur in some subjects and is often life threatening. We describe here a case of fatal JDMS with gastrointestinal perforation. Immunohistochemical examination by antibody against factor VIII seems to be useful for evaluating the pathological basis of vasculopathy in JDMS.

A 13 year old Japanese girl was admitted in April 1994 with high fever, muscle pain, and muscle weakness. She noticed a facial rash for two months before admission. Physical examinations were; blood pressure 135/90 mm Hg, temperature 38°C, and weight 50 kg. She presented with an erythematous rash on her face, neck and arms, heliotropic eruption, Gottron’s sign, and nail fold telangiectasia. Proximal muscular weakness and pain were prominent. Laboratory findings were as follows; stool occult blood negative, leucocyte count 5800/mm3, erythrocyte count 4800 × 103/mm3, thrombocyte count 109 × 103/mm3, and serum C reactive protein value normal. Muscle enzyme examination showed; glutamic oxaloacetic transaminase 294 IU/l, creatinine phosphokinase 5960 IU/l, and lactate dehydrogenase 1469 IU/l. Rheumatoid factor, antinuclear antibody and other autoantibodies were all negative. Electromyogram findings showed short and small motor units. Muscle biopsy specimen showed variation in fibre size and perivascular inflammatory cell infiltration in the connective tissue. JDMS was diagnosed according to the criteria of Bohan and Peter.2 No features of other connective tissue diseases or malignant neoplasms were present.

Intravenous prednisolone (60 mg/day) was started. However, dysphagia occurred and thrombocytopenia (41 × 103/mm3) with increase in platelet associated IgG (268 ng/107platelet) was apparent. Three courses of methylprednisolone pulse therapy (1000 mg/day for three days) and two courses of high dose intravenous immunoglobulin (20 g/day for five days) were prescribed followed by intravenous methotrexate (100 mg/day every two weeks). Although thrombocytopenia and the increase in serum muscle enzyme had improved, abdominal pain, haematemesis, and melaena resulting from multiple gastrointestinal ulcers were noted. Cyclophosphamide pulse therapy (750 mg/day, every two weeks) and plasma exchange were not effective and oesophageal and bowel perforation occurred. After the resection of perforated lesions, peritonitis occurred and she died of massive abdominal haemorrhage seven months after admission (fig 1).

Figure 1

Clinical course. PSL=prednisolone; m-PSL=methylprednisolone; MTX= methotrexate; IVIG=intravenous immunogloburin, CY=cyclophosphamide pulse therapy; PE=plasma exchange; Plt=platelet.

Surgical findings showed three perforated areas (lower oesophagus, ileocaecal region, and gastric antrum). Pathological examination of these lesions showed occlusion of both arteries and veins. The internal elastic lamina of arteries were intact (fig 2A). Intimal hyperplasia was seen in some vessels and some other vessels were occluded by fibrin thrombi with proliferation of the endothelial cells, characterised by positive staining for factor VIII (fig 2B and C). Some vessels showed infiltration of lymphocytes and foamy macrophages to the adventitia and media.

Figure 2

(A) Vascular lesion at the oesophageal perforation. Both the artery (left) and vein (right) were occluded. The internal elastic lamina of the artery was intact, elastica Van Gieson stain. (B). The serial section of figure 2A. Deep blue fibrin obliterated the lumen of the vein, PTAH stain. (C) The serial section of figure 2A and2B. Endothelial cells were stained with anti-factorVIII antibody (F8/86, mouse IgG1, DAKO), immunohistochemical stain for factor VIII. Bar=0.5 mm.

In JDMS, an autoimmune connective tissue disease, the microvasculature is thought to be the fundamental site of pathology. Banker and Victor reported that the earliest pathological changes of vessels were perivascular collections of inflammatory cells, followed by intimal hyperplasia of arteries and veins.1 Vessel lumen may be occluded by thrombi, fibrin or swollen endothelial cells.1-4

In our case the occluded vessels consisted of intimal hyperplasia and fibrin thrombi with proliferated cells, which were stained with anti-factorVIII antibody, an endothelial cell marker.5Although the level of factor VIII related antigen was not measured in this case, a high level of factor VIII related antigen in JDMS has been previously reported by some authors.3 ,6-8 Our immunohistochemical findings suggest the endothelial cell dysfunction in the vasculopathy of JDMS and also suggest that anti-factor VIII antibody is useful for evaluating the pathological basis of vasculopathy in JDMS.

Thrombocytopenia seen in our case was accompanied by an increase in platelet associated IgG, and improved after treatment with prednisolone and intravenous immunoglobulin. This suggested that her thrombocytopenia resulted from autoimmune thrombocytopenia associated with dermatomyositis, which has been reported in only two adult cases.9 ,10 This is the first report of autoimmune thrombocytopenia in JDMS.


We thank Dr H Kobayashi, for advice on dermatology; M Ohara for helpful comments.