J Korean Rheum Assoc.  2000 Sep;7(3):313-322.

A Case of Systemic Sclerosis Sine Scleroderma

Affiliations
  • 1Department of Internal Medicine, Catholic University of Taegu School of Medicine, Taegu, Korea.

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.

Keyword

Systemic sclerosis sine scleroderma, Intestinal pseudoobstruction

MeSH Terms

Antibodies, Antinuclear
Connective Tissue
Decompression
Erythromycin
Esophageal Motility Disorders
Extremities
Humans
Intestinal Pseudo-Obstruction
Octreotide
Scleroderma, Systemic*
Sclerosis
Skin
Antibodies, Antinuclear
Erythromycin
Octreotide
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