Pneumatosis Cystoides Intestinalis with Systemic Sclerosis, Limited Type Resulting in a Poor Prognosis
Department of Dermatology, Kyushu University, Hukuoka, Fukuoka, JapanThe American Journal of the Medical Sciences (Impact Factor: 1.39). 09/2006; 332(2):100-2. DOI: 10.1097/00000441-200608000-00011
Pneumatosis cystoides intestinalis (PCI) is a rare disease characterized by the presence of multilocular intramural clusters of gas in the alimentary tract and has been considered to have a favorable response to conservative treatment. We describe the first case of limited type of systemic sclerosis (SSc) with PCI. A 74-year-old Japanese woman presented with a 4-month history of an unhealed cutaneous ulcer on the right third finger, along with sclerodactyly of bilateral hands. Proximal skin sclerosis was absent. The patient reported acute abdominal pain, and a diagnosis of PCI was established on plain radiography. The patient died of multiple organ failure 5 months after the development of PCI. PCI is rarely complicated with SSc, and all cases previously reported were associated with diffuse SSc. Because PCI is one of the poor prognostic factors of SSc, we should recognize the presence of this condition even in patients with limited cutaneous involvement.
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ABSTRACT: Systemic sclerosis (SSc, scleroderma) is a chronic, connective tissue disease characterized by vascular damage and fibrosis of the skin and internal organs including the gastrointestinal (GI) tract. After skin involvement, the GI tract is the second most commonly involved organ system with the esophagus being the most frequent segment involved. Involvement of the stomach, small intestine, colon, and anorectum is less common but may lead to severe complications and debility. Ultimately, over 80% of individuals with scleroderma will experience a change in normal GI function (Szamosi S, Szekanecz Z, Szucs G. Gastrointestinal manifestations in Hungarian scleroderma patients. Rheumatol Int. 2006;26:1120–4; Weston S, Thumshirn M, Wiste J, et al. Clinical and upper gastrointestinal motility features in systemic sclerosis and related disorders. Am J Gastroenterol. 1998;93:1085–9).
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