A Case of Systemic Sclerosis Sine Scleroderma / 대한류마티스학회지

ABSTRACT

Systemic sclerosis (SSc) is a generalized connective tissue disorder of unknown etiology. Clinically, there is a broad spectrum of disease ranging from widespread severe skin thickening to skin thickening limited to the distal extremities. In rare cases of systemic sclerosis, no cutaneous change only with internal organ involvement has been reported, which is called ?ystemic sclerosis sine scleroderma (ssSSc)?. We describe a patient with Raynaud? phenomenon, who showed intestinal pseudoobstruction as a presenting symptom but did not show any skin change. She had also an esophageal motility disorder, but other organ involvement was not evident. Antinuclear antibody was positive. Her obstruction symptoms were improved by decompression by nasogastric tube and pharmaceutical treatment with erythromycin and octreotide.