A dermatomyositis and scleroderma overlap syndrome with a remarkable high titer of anti-exosome antibodies

Abstract

An overlap syndrome of dermatomyositis and scleroderma is reported. The case corresponded to a 27-year-old female with a clinical picture of 14 months evolution, characterized by proximal muscles weakness, erythematous rash in wrists, knees, ankles, Gottron sign, heliotrope periorbital rash and dysphagia. A muscle biopsy was compatible with dermatomyositis; meanwhile the skin biopsy was compatible with scleroderma. Muscle enzymes were increased. Interestingly, the antinuclear antibody determination in HEp-2 cells was positive with a remarkable titer of 81,920 exhibiting a nucleolar pattern. Anti-Jo1 antibody was negative, but anti-PM/Scl-100 positive. The patient received methylprednisolone and cyclophosphamide pulses, with gradual improvement. Present report constitutes a case of overlap dermatomyositis-scleroderma syndrome, with anti-PM/Scl autoantibodies (anti-exosome). The remarkable of this case was the exceptional high antinucleolar antibody titer.